Common Pathologies of the Brain: Preparing for ICD-10-CM/PCS © Irene Mueller EdD, RHIA May 16, 2013

Objectives Review Common Brain Pathologies for ICD-10-CM/PCS Coding Circulatory pathologies affecting brain Neurological pathologies affecting brain ICD-10-CM/PCS Terminology (Integrated) Did you Complete the PRE-Test?

Overlap of Circulatory and Nervous Systems Circulatory pathologies affecting brain Neurological pathologies affecting brain

Cerebrovascular Diseases Affect Cerebral & Precerebral blood vessels Atherosclerosis Aneurysm Stroke Non-traumatic hemorrhage Infarction/obstruction Occlusion/Stenosis

Atherosclerosis Build up of waxy plaque on inside of blood vessels (inner layer only), can block blood flow Gk, athere = gruel, skleros = hard Atherosclerosis Type of arteriosclerosis Arteriosclerosis = general term for hardening of arteries (inner/middle layers of artery wall) Arteriosclerosis can occur in several forms, including atherosclerosis Plaque composed of fatty substances, cholesterol, waste products from cells, calcium, and fibrin

Atherosclerosis Progression Inner artery wall thickens, Artery's diameter reduced, Blood flow and oxygen delivery are decreased (stenosis, stricture) Plaques can rupture or crack open Sudden formation of blood clot (thrombus, embolus) Complete blockage Stroke if located in precerebral/cerebral arteries

Risk factors for Atherosclerosis Response to endothelial damage High cholesterol, High blood pressure, & cigarette smoking All 3 = 8X more likely Physical inactivity, diabetes, & obesity High levels of Homocysteine Lipoproteins Low levels of HDL Heredity Parents have AD, atherosclerosis, or stroke early African-Americans (HTN) Sex – Males 45+, Women 55+ Equal risk after 60

Aneurysm Localized, blood-filled dilation of blood vessel caused by disease/weakening of vessel wall Often incidental finding Berry aneurysm small (size of berry) saccular aneurysm of cerebral artery, usu. at junction of vessels in Circle of Willis Narrow opening into artery http://www.nhlbi.nih.gov/health/health-topics/topics/arm/printall-index.html

Cerebral Aneurysms 3 to 5 million people in US have cerebral aneurysms Most do NOT produce symptoms 0.5 - 3 % of people may suffer from bleeding Usually develop at arteries’ branching points Caused by constant pressure from blood flow Often enlarge slowly/become weaker One aneurysm = 15 to 20 % chance of more aneurysms

Cerebral Aneurysm Subarachnoid hemorrhage due to rupture of cerebral aneurysm - most common ages 20 to 60 Slightly more common in women than men Precipitators of aneurysm rupture Heavy lifting/straining can cause pressure to rise in brain Strong emotions (upset/angry) can raise blood pressure Blood “thinners” (warfarin, etc.) Some meds/prescription drugs (also stimulant diet pills such as ephedrine and amphetamines) Harmful drugs like cocaine can cause aneurysms to rupture and bleed

Other precursors of aneurysms Other blood vessel disorders Fibromuscular dysplasia, cerebral arteritis or arterial dissection VERY unusual Infections Drugs (amphetamines/cocaine) that damage brain blood vessels Direct brain trauma from accident Elderly = Falls Young = MVAs

Aneurysms Size Shape Location Small = < 5 mm (1/4 inch) Medium = 6–15 mm (1/4 to 3/4 inch) Large = 16–25 mm (3/4 to 1 inch) Giant = > 25 mm (1 inch+) Shape Saccular (sack-like) with a well-defined neck Saccular with wide neck Fusiform (spindle shaped) without a distinct neck Location Cerebral aneurysm –usu. located along major arteries deep within brain structures Anterior OR posterior circulation

Stroke AKA - Brain attack, CVA, CVI (Cerebrovascular Insult), Infarct, Apoplexy Sudden death of brain cells in localized area due to inadequate blood flow Single most costly disease 3.4 million women in the US living w/disabilities resulting from stroke

Controllable Risk Factors for Stroke Lifestyle Tobacco Use & Smoking Alcohol Use Physical Inactivity Obesity Medical High Blood Pressure Atrial Fibrillation High Cholesterol Diabetes Atherosclerosis/Circulation Problems Migraine esp. women <55 Sickle Cell Disease Sleep Apnea TIA

Uncontrollable Risk Factors for Stroke 55 + Male (Female in later years) African American, Hispanic or Asian/Pacific Islander Family Hx of stroke or TIA Fibromuscular Dysplasia PFO (congenital heart defect) Low birth weight Study showed weight < 5 lb., 8 oz. at birth = 2X stroke risk as weight of 8 lb., 13 oz +

Stroke Signs and Symptoms SUDDEN (Men & Women) Numbness/weakness of face, arm or leg – especially one side of body Confusion, trouble speaking or understanding Trouble seeing in one or both eyes Trouble walking, dizziness, loss of balance or coordination Severe headache w/no known cause Women may report unique stroke symptoms SUDDEN Face and limb pain General weakness Palpitations Chest pain Hiccups Nausea SOB

Stroke S&S Common stroke symptoms Loss of balance or coordination Dizziness (Vertigo – spinning sensation) Slurred speech (Dysphasia) Loss of previous ability to speak/understand spoken or written language (Aphasia) NOT Aphagia Dysphagia/Aphagia (poor, NO swallowing) Paralysis, numbness, or weakness on one side of the body (Hemiparesis, Hemiplegia) Blurred, double, or blackened vision (Diplopia) Sudden, severe headache (Cephalalgia, encephalgia)

for Different Types of Stroke Surviving a Stroke 30-Day Death Rates for Different Types of Stroke Ischemic Stroke Hemorrhagic Stroke Women 7% 30% Men 8% 35% 45 to 64 y-o 8% to 12% 37% 65 y-o + 45%

Surviving a Stroke PT Sex/Age w/i1 yr w/I 5 yrs Female DEATH (40+) 24% 51% Male DEATH 21% 47% Female 2nd Stroke 22% Male 2nd Stroke 13% Female (60-69) AVG Survival 7.4 years Male (60-69) AVG Survival 6.8 years Female (80+) 3.1 years Male (80+) 1.8 years

Surviving a Stroke 50% to 70% of stroke survivors eventually regain functional independence 15% to 30% permanently disabled 65 +y-o survivors of ischemic stroke had disabilities 6 mos. post-stroke 50% hemplegia 35% depressive symptoms 30% couldn’t walk w/o assistance 26% in nursing home 26% dependent in ADLs 19% aphasia

Types of Strokes Ischemic (80 +%) Hemorrhagic (More Severe) Thrombus (Clot) Embolus (MOVING Clot) Occlusion Stenosis Hemorrhagic (More Severe) Blood vessel in brain leaks or ruptures Common types include Intracerebral Subarachnoid Extradural/epidural Subdural hemorrhages http://www.nhlbi.nih.gov/health/health-topics/topics/stroke/printall-index.html

Non-Traumatic Hemorrhage AKA – Hemorrhagic Stroke Subarachnoid Outside brain Between Arachnoid and Pia mater Intracerebral Within brain Causes Intracranial aneurysm (most common) AV malformation, Tumor, Infection

Cerebral Infarction AKA – Ischemic stroke Causes Blood supply to brain site is interrupted or severely reduced Brain tissue is starved of O2 and nutrition Ischemia Necrosis Causes Thrombus or embolus blocks blood flow in cerebral OR precerebral arteries

Occlusion vs Stenosis Occlusion L, occludere, to close up blockage in a canal, vessel, or passage of body; state of being closed AKA – obstruction of precerebral/cerebral arteries Stenosis [Gr.] stricture; AKA - arctation, coarctation, stricture Abnormal narrowing or contraction of duct or canal Narrowing of artery can decrease blood supply to brain Increases risk of CVA

Stroke vs. Occlusion/Stenosis Has blood supply loss caused DEATH of brain cells/tissue? Evidence of Death of brain cells = Speech/language deficits Monoplegia Hemiplegia Other cognitive or functional deficits Occlusion w/ or w/o cerebral infarction Classified separately based on IF infarct results from occlusion

CVA vs TIA Symptoms similar, BUT TIA symptoms usually resolve w/in 24 hours AKA Little/Mini stroke – Most common cause is embolus Precursor to Stroke in many cases Signs/Symptoms include: Numbness/weakness in face, extremity - usu one side Confusion; Difficulty walking Difficulty speaking OR understanding Vision change in one/both eyes Dizziness; Loss of balance/coordination RIND - Reversible Ischemic Neurologic Deficit CVA/w deficits such as hemiplegia, dysphagia, slurred speech lasting longer than TIA May persist for as long as six months BUT eventually resolve

Specific Effects on Brain/Sequelae Sequela/Sequelae are residual effects or conditions produced after acute phase of illnes/injury has ended NO time limit on when sequela code can be assigned Residuals may be apparent early on such as in cerebral infarction, or they can occur months or years later Cerebrovascular disease sequelae Include aphasia, dysphagia, monoplegia, or hemiparesis Arise from any condition classifiable to categories I60-I67 Sequalae ICD-10-CM codes REQUIRE type of Cerebrovascular Disease (has unspecified option)

Cognitive Sequalae Memory Loss Ability to remember names/faces/shapes can be compromised Difficulty learning new information or skill Difficulty organizing thoughts/perform sequential tasks (Frontal lobe) Agnosia - Inability to recognize specific familiar object, (e.g., own body hand, face, shape, or sound) Ex: "finger agnosia" due to stroke in dominant language parietal lobe, pt can easily recognize/name car or TV, but not own thumb Agraphia - Loss of writing ability resulting from damage to language areas of brain After stroke often incomplete, many stroke survivors can rapidly re-learn to write some words/sentences

Language Sequalae Alexia - injury prevents people from being able to read, but not to write AKA: word blindness, pure word blindness, text blindness, or visual aphasia Agraphia - Loss of writing ability resulting from damage to language areas of brain After stroke, agraphia often incomplete Many stroke survivors rapidly re-learn to write some words/sentences

Movement Sequalae Apraxia Ataxia Dystonia Weakness or paralysis on side of body opposite stroke Unmasking of primitive reflexes (instinctive sucking, grasping, & groping Compulsive mimicking of facial gestures made by others Compulsive repetition of movement (motor perseveration)

Types of Paralysis Distribution of weakness = Important clue to location of nerve damage "-plegia," = Gk, - "stroke" Classified by region Monoplegia - only one limb Diplegia - same body region on both sides of body (both arms, for example, or both sides of face) Hemiplegia - one side of body Paraplegia - both legs and trunk Quadriplegia (Tetra-) - all four limbs and trunk

Dominance and Paralyses Right-hand patient = right-side dominant Left-handed patient = left-hand dominant When affected side is documented, but NOT specified as dominant/non-dominant AND classification system does not indicate default Code selection For ambidextrous patients, default = dominant Left side affected, default = non-dominant Right side affected, default = dominant

Dominance Example 1 Pt admitted for outpatient PT therapy for monoplegia of left leg affecting non-dominant side due to spontaneous subarachnoid hemorrhage 2 weeks ago I69.044, Monoplegia of lower limb following nontraumatic subarachnoid hemorrhage affecting left non-dominant side

Dominance Example 2 A patient presents for follow-up 1 month after suffering cerebral infarction Dr. notes pt suffers from right-sided hemiplegia due to infarction & recommends continued PT and OT I69.351 Hemiplegia and hemiparesis following cerebral infarction affecting right dominant side

Dominance Example 3  Pt presents w/new R middle cerebral artery embolism & infarction causing aphasia Pt suffered ruptured cerebral aneurysm one year prior w/residual oral phase dysphagia I63.411 Cerebral infarction due to embolism of right middle cerebral artery *I69.320 Aphasia following cerebral infarction I69.091 Dysphagia following nontraumatic subarachnoid hemorrhage R13.11 Dysphagia, oral phase *Sequalae = neurologic deficits that persist after initial onset of conditions

Hemiparesis Example 65-yo female seen for Tx of unstable angina. She has Hx of 2-vessel CABG about 18 months ago. Recent cardiac catheterization shows continued evidence of coronary atherosclerosis, but grafts are patent. Also, pt suffered CV Infarction 3 yrs ago, which resulted in R-sided (dominant) hemiparesis I25.110 Angina (attack) (cardiac) (chest) (heart) (pectoris) (syndrome) (vasomotor), with atherosclerotic heart disease – see Arteriosclerosis, coronary (artery), native vessel with angina pectoris, unstable I69.351 Hemiparesis – see Hemiplegia, following, cerebrovascular disease, cerebral infarction Z95.1 Status (post), aortocoronary bypass

Hemiparesis Example I69.35 Hemiplegia and hemiparesis following cerebral infarction I69.351 Hemiplegia and hemiparesis following cerebral infarction affecting right dominant side I69.352 Hemiplegia and hemiparesis following cerebral infarction affecting left dominant side I69.353 Hemiplegia and hemiparesis following cerebral infarction affecting right non-dominant side I69.354 Hemiplegia and hemiparesis following cerebral infarction affecting left non-dominant side I69.359Hemiplegia and hemiparesis following cerebral infarction affecting unspecified side

Sensory Sequalae Strokes in sensory cortex of parietal lobe can cause profound numbness Astereognosis = inability to identify object by sense of touch AKA tactile agnosia

Speaking Sequalae Alogia - Complete lack of speech Aphonia - Complete speechlessness resulting from inability to produce normal sounds due to organic (–eg, laryngeal disease or mental cause) Dysphonia - Any disorder of phonation affecting voice quality/ability to produce voice Apraxia: Inability to execute voluntary motor movement despite being able to demonstrate normal muscle function NOT lack of understanding or physical paralysis Problem in brain cortex

Speaking Sequalae Traumatic injury Oxygen deprivation during stroke Aphasia - condition characterized by partial or total loss of ability to communicate verbally/written words Pt may have difficulty speaking, reading, writing, recognizing names of objects, or understanding what other people say Caused by brain injury Traumatic injury Oxygen deprivation during stroke Brain tumor Alzheimer Infection, like encephalitis Temporary or permanent Does NOT include speech impediments caused by loss of muscle control Dysphasia - partial or complete impairment of the ability to communicate resulting from brain injury - used more in Europe

Aphasia Example Gentleman seen in clinic for followup of previous stroke. He had cerebrovascular infarction 6 months ago, which left him with aphasia and left-sided hemiparesis on his non-dominant side. Pt is referred to Outpt Rehab for ST, PT, and OT. I69.354 Hemiparesis – see Hemiplegia, following, cerebrovascular disease, stroke I69.320 Aphasia, following cerebrovascular disease, cerebral infarction

Swallowing Sequalae Aphagia -condition characterized by loss of ability to swallow Result of organic disease or psychologic causes such as cerebrovascular accident or anxiety Dysphagia - difficulty in swallowing Numerous underlying causes Stroke/other neurologic conditions Local trauma and muscle damage Tumor/swelling partially obstructing passage of food Ranges from mild discomfort, such as feeling a lump in throat, to severe inability to control muscles needed for chewing and swallowing

Dysphagia Example Acute CV Infarction due to embolism of left cerebellar artery with dysphagia and right hemiplegia. I63.442 Infarct, infarction, cerebellar – see Infarct, cerebral. (See also Occlusion, artery, cerebral, or precerebral, with infarction). Occlusion, occluded artery, cerebellar (anterior inferior) (posterior inferior) (superior) with infarction, due to, embolism R13.10 Dysphagia G81.91 Hemiplegia

Dysphagia Example I63 Cerebral infarction Includes: occlusion and stenosis of cerebral and precerebral arteries, resulting in cerebral infarction Use additional code, if applicable, to identify status post administration of tPA (rtPA) in different facility within last 24 hours prior to admission to current facility (Z92.82) Excludes1 sequelae of cerebral infarction (I69.3-)

Dysphagia Example I63.4 Cerebral infarction due to embolism of cerebral arteries I63.44 Cerebral infarction due to embolism of cerebellar artery I63.441 Cerebral infarction due to embolism of right cerebellar artery I63.442 Cerebral infarction due to embolism of left cerebellar artery I63.449 Cerebral infarction due to embolism of unspecified cerebellar artery I69.391 Dysphagia following cerebral infarction Use additional code to identify the type of dysphagia, if known (R13.1-)

Dysphagia Example R13.1 Dysphagia R13.19 Other dysphagia Code first, if applicable, dysphagia following cerebrovascular disease (I69. with final characters -91) Excludes1: psychogenic dysphagia (F45.8) R13.19 Other dysphagia Cervical dysphagia Neurogenic dysphagia

Frontal Lobe Sequalae Motor Weakness or paralysis on side of body opposite stroke Unmasking of primitive reflexes (instinctive sucking, grasping, & groping Compulsive mimicking of facial gestures made by others Compulsive repetition of movement (motor perseveration) Abulia - Lack of motivation/desire to perform task In stroke, most often due to damage to frontal lobe Ex:s. Stroke survivor fails to move arm/leg, even though part of brain required to carry out movement is intact Apraxia of gait Urinary incontinence

Frontal Lobe Sequalae Cognition and Intellect Behavior and Personality Lack of initiative, vacillation, mood changes & inattentiveness Difficulty solving problems (goal-directed behavior) in different areas of cognition including psycholinguistic, constructive, logical, and arithmetical Behavior and Personality Profound lack of initiative and motivation Spontaneous expression of socially inappropriate remarks Irritability Carelessness and apathy Inappropriate & seemingly random persistence & repetition of certain behaviors

Frontal Lobe Sequalae Speech and Language Broca aphasia - stroke affects dominant language hemisphere http://en.wikipedia.org/wiki/File:BrocasAreaSmall.png

Types of Aphasia Broca’s Aphasia – AKA, Motor Aphasia Production of language is affected, (speaking) But other aspects of language are mostly preserved Usu. prevents forming own intelligible words/sentences, but still ability to understand others. Some aphasics can say few words - telegraphic speech Often w/other impairments –hemiparesis/hemiplegia on R side of body, alexia and agraphia Wernicke’s Aphasia Pts can’t understand others’, or even their own speech Speech is incomprehensible, but pt feels being understood. (Anosagnosia) Ex: “My door sat through the lamp in the sky.” (Logorrhea) With time, Wernicke’s aphasics may know others can’t understand them - might become angry, paranoid, & depressed Global Aphasia Involves both areas Pts can’t understand spoken language or speak Some pts can still communicate by using written language

Parietal Lobe Sequalae Visual Symptoms Homonimous quadrantanopsia AKA quadrantanopsia; quadrantic anopsia; quadrantic hemianopia Nerve fibers carry information from lower parts of visual field Travel through parietal lobes to occipital lobe When damaged by parietal strokes causes vision in lower quarter of side opposite stroke to be lost Spatial Dysperception Difficulty interpreting visual information in surroundings, such as length, depth, & size of objects Hand-Eye Incoordination Difficulty bringing hand to spot where looking EX: Pt looks at coffee mug on table, means to pick it up, but hand overshoots, and can’t grab mug. Inability to visually scan surroundings Has full eye movement, but prevents seeing objects, people, and other visual stimuli presented in peripheral vision

Parietal Lobe Sequalae Abnormalities of Self-Perception Hemineglect: parietal strokes in non-dominant language side of brain tendency to completely ignore opposite side of body Ex: Hemiplegia due to stroke affecting both motor& sensory cortices Pts ignore fact that one side of body completely paralyzed, but also can't recognize own body parts on that side So fail to shave or wear lipstick on side affected by stroke Finger agnosia Selective inability to name fingers Right-left confusion Profound inability to differentiate R from L Difficulty w/reading, writing, and math

Parietal Lobe Sequalae Sensory Symptoms Sensory loss Strokes in sensory cortex (in parietal lobe) can cause profound numbness Astereognosis Other Inattentiveness Apathy Dullness

Parietal Lobe Sequalae Constructional apraxia Inability to build whole from its single parts Ex: inability to assemble simple 4-piece puzzle Eye-opening apraxia Inability to open eye despite will and physical ability Ideomotor apraxia Inability to mimic learned motor task/behavior (combing hair) Pt is unable to pantomime how to comb Results from damage to dominant-side parietal lobe

Temporal Lobe Sequalae Language Wernicke aphasia Word-finding difficulties Pure word deafness Can’t hear words, but can recognize bells, buzzers, etc. Can’t write when asked, but can write spontaneously Transcortical aphasia Uncommon Amnestic dysnomia Difficulty retrieving names for things/people

Temporal Lobe Sequalae Hearing Hearing loss Mild when one temporal lobe affected Complete deafness possible when both affected , but very rare Auditory agnosia Difficulty recognizing combinations of sounds such as songs, musical tones, and complex conversations Auditory verbal agnosia = pure word deafness Auditory illusion Aberrant perception of normal sounds - feel unusual, strange, repeated, or loud Auditory hallucinations Hearing sounds NOT there Complex = sound of song on radio Simple = whistles or siren May occur w/visual hallucinations Pts may/may not realize these are hallucinations

Temporal Lobe Sequalae Memory, Emotion and Behavior Loss of short or long term memory Fits of rage Violent/aggressive behavior Placidity Lack of interest Abnormally enhanced sexuality Other Vertigo (type of balance problem) Abnormal perception of time Feelng that time stands still /goes extremely quickly Intermittent loss of sense of year, season or month Disturbances of smell and taste Seizures

Occipital Lobe Sequalae Stroke Affecting Entire Occipital Lobe on One Side Homonomous Hemianopia – Pt not able to see objects on opposite side of body Stroke Affecting Occipital Pole – 'Central Vision Defect‘ Central Vision = Seen at center of visual field when looking straight ahead Large blind spot in middle of visual field on affected side Pt looking straight ahead at someone’s face May not be able to see nose, upper lip, & lower half of eye on affected side But could see shoulder and top of head on that side

Occipital Lobe Sequalae Stroke Affecting Occipital Lobes on Both Sides – 'Cortical Blindness‘ Blindness Also sometimes also suffer from visual anosagnosia AKA Anton syndrome Other Visual Illusions - Distortion of movement, form, size or color in visual field Visual Hallucinations Visual Agnosias Balint Syndrome Prosopagnosia - Inability to recognize identity of familiar faces Alexia W/O Agraphia

http://en. wikipedia. org/wiki/File:Dean_Franklin_-_06. 04 http://en.wikipedia.org/wiki/File:Dean_Franklin_-_06.04.03_Mount_Rushmore_Monument_%28by-sa%29-3_new.jpg

Balint Syndrome Group of 3 symptoms occurring together Result of stroke at border of parietal and occipital lobes Inability to voluntarily look around in space Inability to grab object while looking at object, due to dis-coordination of eye/hand movements Tendency to only see one object in visual field at one time

Cerebellum Sequalae Difficulty w/ coordination & posture, as well as ability to execute fluid movement Common effects of cerebellar strokes include Inability to walk, problems with coordination and balance (ataxia) Dizziness Headache Nausea Vomiting

Brain Stem Sequalae Common effects of stroke in brain stem include problems with Breathing and heart functions Body temperature control Balance and coordination Weakness or paralysis in all four limbs Chewing, swallowing, and speaking Vision Coma Death is common

Current CV Infarction Example Acute cerebral infarction, thrombosis of left anterior cerebral artery with residual right-sided hemiplegia I63.322 Infarct, infarction, cerebral – (see also Occlusion, artery, cerebral or precerebral, with infarction). Occlusion, artery, cerebral, anterior, with infarction, due to, thrombosis OR Infarct, infarction, cerebral, due to thrombosis, cerebral artery. G81.91 Hemiplegia

Current CV Infarction Example G89.9 Hemiplegia, unspecified G89.90 Hemiplegia, unspecified affecting unspecified side G89. 91 Hemiplegia, unspecified affecting right dominant side G89. 92 Hemiplegia, unspecified affecting left dominant side G89. 93 Hemiplegia, unspecified affecting right nondominant side G89. 94 Hemiplegia, unspecified affecting left nondominant side

Neurological Pathologies

Some diseases of brain result of unnatural deaths of neurons Parkinson’s disease Neurons producing dopamine die in basal ganglia Causes difficulty initiating movement Huntington’s disease Genetic mutation causes over-production of glutamate Kills neurons in basal ganglia Uncontrollable twisting/writhing

Deaths of neurons, cont. Alzheimer’s disease – Unusual proteins build up in & around neurons in neocortex & hippocampus Control memory, causing inability to remember or do everyday tasks Blows to brain or stroke damage Can kill neurons outright OR slowly starve of oxygen and nutrients Spinal cord injury Can disrupt communication between brain & muscles Neurons lose connection to axons located below site of injury; which may still live, but lose ability to communicate

Infectious Pathologies of Brain G00-G09

Encephalitis and encephalomyelitis Inflammation of encephalon (part of nervous system within cranium (brain)) Usually viral West Nile encephalitis Severe form of infection Neuroinvasive affecting brain 20 % mild/moderate signs/symptoms <1 % encephalitis/meningitis/poliomyelitis Headache, high fever, stiff neck, stupor, coma, paralysis

Herpes Zoster Varicella Virus Virus lies dormant in nerve cells Chicken Pox, can cause Herpes Zoster (Shingles) Virus lies dormant in nerve cells Reactivated = burning, itching, tingling, sensitive skin, then rash and blisters (usually one area on one side of body - dermatomes) Herpes zoster encephalitis Herpes zoster meningitis

Meningitis Inflammation of membranes covering brain & spinal cord Usu/ dura mater and/or arachnoid AKA arachnoiditis AKA pachymeningitis Bacterial, Viral, Non-infectious Acute or Chronic

Meningitis Example Pt admitted with high fever, stiff neck, chest pain, and nausea. Lumbar puncture results were positive for meningitis. Chest X-ray showed pneumonia. Sputum culture grew pneumococcus. Pt tx with antibiotics. Dx: Pneumococcal meningitis and pneumococcal pneumonia. G00.1 Meningitis, pneumococcal J13 Pneumonia, pneumococcal, (broncho) (lobar)

Meningitis Example Excludes1: bacterial: G00.1 Pneumococcal meningitis G00 Bacterial meningitis, NEC Includes: bacterial arachnoiditis bacterial leptomeningitis bacterial meningitis bacterial pachymeningitis Excludes1: bacterial: meningoencephalitis (G04.2) meningomyelitis (G04.2) G00.0 Hemophilus meningitis Meningitis due to Hemophilus influenzae G00.1 Pneumococcal meningitis Pneumonia (acute) (double) (migratory) (purulent) (septic) (unresolved) J18.9 -- pneumococcus J13 J13 Pneumonia due to Streptococcus pneumoniae Bronchopneumonia due to S. pneumoniae Code first: associated influenza, if applicable (J09.X1, J10.0-, -J11.0-) Code also: associated abscess, if applicable (J85.1) Excludes1: congenital pneumonia due to S. pneumoniae (P23.6) lobar pneumonia, unspecified organism (J18.1)

Systemic Atrophies G10-G13

Systemic Atrophies Motor neuron diseases Affect Muscle control/strength Many have Hereditary component

Medical Terminology Atrophy Progressive, acquired decrease in size of normally developed cell, tissue, or organ May result from decrease in cell size, # of cells, or both L, atrophia, Grk, from atrophos ill-fed Ataxia Loss of ability to coordinate muscular movement, most frequently resulting from disorders in brain/spinal cord AKA Dyssynergia, Incoordination Palsy Complete or partial muscle paralysis, often accompanied by loss of sensation & uncontrollable body movements or tremors Sclerosis Condition characterized by hardening of tissue resulting from any of several causes AKA Induration

Muscular atrophy Decrease in size and wasting of muscle tissue Muscles that lose their nerve supply can atrophy; simply waste away http://www.nlm.nih.gov/medlineplus/ency/imagepages/9680.htm

Amyotrophic lateral sclerosis Disease of motor neurons Upper Motor Neurons - Nerve cells reaching from brain to spinal cord Spasms & tonic limbs Abnormal reflexes Lower Motor Neurons –from spinal cord to peripheral nerves that control muscle movement Muscle wasting Fasciculations (Twitching) AKA - Lou Gerhrig’s Disease, motor neuron disease Cause Unknown Prognosis – 50% of pts die w/in 3 years 80% die w/in 5 years 10 % Live more than 8 years http://en.wikipedia.org/wiki/File:Gehrig_cropped.jpg

Hereditary spastic paraplegia Hereditary spastic paraplegia (HSP) - group of inherited disorders characterized by progressive weakness & spasticity (stiffness) of legs AKA familial spastic paraparesis (FSP) 20 genes responsible for several forms of HSP now identified Variable prosnoses Uncomplicated, Complicated

Huntington Disease Inherited degenerative disorder of cerebral cortex & corpus striatum AKA Huntington Chorea, hereditary chorea, chronic progressive chorea; degenerative chorea; degenerative chorea; Huntington disease, Woody Guthrie's disease George, U.S. physician, 1850-1916 described in 1872 HD gene located on short arm of chromosome 4 Dominant gene Affects males and female Can be inherited from either parent (also w/HD). 50% chance of passing it to each Child Begins usu. in 30s-50s

Huntington Disease S&S 3 categories Motor or movement symptoms Dystonia Sustained abnormal postures, including facial grimaces, twisted neck, or arched back Chorea Involuntary jerking, twisting or writhing motions Slowness of voluntary movements Can’t control speed or force of movements Can’t initiate movement Slowed reactions Difficulty speaking and swallowing Localized/generalized weakness Impaired balance ability Rigidity, especially in late-stage disease

Huntington Disease S&S Personality and behavioral changes Depression, irritability, anxiety and apathy May become impulsive, aggressive or socially withdrawn Cognitive decline Loss of ability to plan and execute routine tasks Slowed thought Impaired or inappropriate judgment. Short-term memory loss usually occurs, although long-term memory is usually not affected Pt w/ late-stage HD Usu. retains knowledge of environment Recognizes family members or other loved ones

Extrapyramidal and Movement Disorders G20-G26

Parkinson Disease vs. Parkinsonisms Parkinsonism = clinical definition of variety of different underlying pathologies causing Parkinson’s-like symptoms - slowing of movement, tremor, rigidity/stiffness, & balance problems  Several disorders produce symptoms referred to as Parkinsonisms - Parkinson disease is ONLY ONE Typical Parkinson patient has Lewy bodies in brain neurons When patient given dopamine replacement therapy (e.g., Sinemet), symptoms go away Parkinsonisms (AKA atypical Parkinson) – add’l symptoms do NOT respond to dopamine replacement therapy 

Parkinson Disease James Parkinson, English physician and paleontologist (1817) AKA - Parkinson's, idiopathic parkinsonism, primary parkinsonism, PD, hypokinetic rigid syndrome/HRS, or paralysis agitans "Involuntary tremolous motion, with lessened muscular power, in parts not in action and even when supported; with a propensity to bend trunk forewards, and to pass from a walking to a running pace: senses and intellect being uninjured."

Parkinson Disease http://www.umm.edu/patiented/articles/what_parkinsons_disease_what_causes_it_000051_1.htm http://students.cis.uab.edu/kelseycp/Parkinson%27s.html

Parkinson S&S Tremor – Resting, Pill-Rolling Slowed movement (Bradykinesia) Over time, reduced ability to move/slow movement Steps may become shorter, difficult to get out of chair, shuffling walk Rigid muscle - any body part, limiting ROM & causing pain Impaired posture and balance Posture may become stooped, may have balance problems Loss of automatic movements Decreased ability to perform unconscious movements, including blinking, smiling or swinging arms when walking, may no longer gesture when talking Speech changes - may speak softly, quickly, slur or hesitate before talking, more monotonous, less inflections Writing changes - may appear small and become difficult

Primary vs. Secondary Parkinson Vascular disease Infectious and postinfectious Postencephalitic – Neurosyphilis - AIDS Toxins Manganese Cyanide Methanol Carbon monoxide 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) Pesticides Medications Neuroleptics Metoclopramide Dopamine-depleting agents (reserpine) Methyldopa Lithium Calcium-channel blockers Valproic acid Fluoxetine Primary (75%) Idiopathic Parkinson's disease No cause determined RARE Causes of 2ndary Metabolic Hypoparathyroidism w/ basal ganglia calcification Hypo/Hyperthyroidism Miscellaneous Repeat trauma (notably from boxing) Structural lesions Tumors Infarctions Hydrocephalus

Parkinson helper dogs Parkinson pts also face 'freezing' Feet freeze in place, but rest of body keeps moving, causing a fall – Some pts tend to become sedentary, reluctant to move, and reclusive Parkinson's helper dogs are trained to identify when person with Parkinson's is 'freezing' If dog touches pt’s foot, it breaks freeze and pt can continue walking Medical experts do not know why this works Dogs are taught to prevent pts falling by counterbalancing & helping regain footing IF pt does fall, dog can help the person up

Parkinsonism Example Pt taking Haloperidol as prescribed for paranoid schizophrenia. Seen for change in facial expressions and stiffness in arms and legs. Dx: 2ndary Parkinsonism due to Haloperidol. G21.11 Parkinsonism (idiopathic) (primary), secondary, due to drugs, neuroleptic T43.4X5A Refer to Drug and Chemical Table, Haloperidol, adverse effect F20.0 Schizophrenia, paranoid (type)

Parkinsonism Example G21 Secondary parkinsonism Excludes1: dementia with Parkinsonism (G31.83) Huntington's disease (G10) Shy-Drager syndrome (G90.3) syphilitic Parkinsonism (A52.19) G21.1 Other drug-induced secondary parkinsonism G21.11 Neuroleptic induced parkinsonism Use additional code for adverse effect, if applicable, to identify drug (T43.3X5, T43.4X5, T43.505,T43.595) Neuroleptic = Major Tranquilizer Haloperidol fits this category Also: Drug Table sends to this

Break Time Fluid Exchanges Ryder Dennehy Ryder Dennehy

Episodic and Paroxymal Disorders G40-G47

Epilepsy Caused by Surge of electrical signals in all/part of brain Seizure manifestations/duration vary widely Convulsions, LOC, blank staring, jerky movements Few seconds to several minutes Epilepsy dx = 2+ incidents of unprovoked seizure Many different types of epileptic seizures 30+, one pt may have several types of seizures

Categories of epileptic seizures Localization-related AKA focal or partial Specific area of one hemisphere Simple = still conscious Complex = Consciousness impaired/LOC May spread to become generalized Auras Generalized Both hemispheres affected from start of seizure Tonic clonic (grand mal); Myoclonic; Absence (petit mal); and Atonic Add’l definition based on Intractable or Not w/wo status epilepticus

Epilepsy terminology Specificity for Seizures of localized onset Complex partial seizures Intractable -Dr. must document w, w/o AKA pharmacoresistent , pharmacologically resistant, treatment resistant, refractory (medically), and poorly controlled (acceptable for coding Intractable) Status epilepticus -Dr. must document w, w/o Recurrent or continuous for 30+ minutes Emergency - Can be life-threatening

Epilepsy Terminology Juvenile myoclonic (JME) Tonic clonic (grand mal) Common – 5% of epileptics Begins in childhood/adolescence Multiple Identified genes increase risk GABRA1 & EFHC1 + others Seizures more likely w/sleep deprivation/alcohol use Tonic clonic (grand mal) Tonic = Stiffening; Clonic = Jerking of limbs and face Myoclonic Rapid, brief contractions of muscles Absence (petit mal) Lapse of Awareness/staring Few seconds Atonic Loss of muscle tone Falls, possible injuries

Epilepsy Documentation for ICD-10 Ex: Pt w/ localization-related (focal) (partial) epilepsy and epileptic syndromes with complex partial seizures, without mention of intractable epilepsy ICD-10-CM has two possible codes G40.201, localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with complex partial seizures, not intractable, with status epilepticus G40.209, localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with complex partial seizures, not intractable, without status epilepticus If your Drs don’t note w/ or w/o status epilepticus, start asking for it now If your Drs don’t note Intractable/Not intractable, start asking for it now

Epilepsy Example 1 62 y-o seen for Tx of recurrent seizures ICD-10-CM 2013 Index Seizure(s) (see also Convulsions) R56.9 recurrent G40.909 G40.909 Epilepsy, unspecified, not intractable, without status epilepticus Epilepsy NOS Epileptic convulsions NOS Epileptic fits NOS Epileptic seizures NOS Recurrent seizures NOS Seizure disorder NOS

Epilepsy Example 2 62 y-o seen for Tx of seizure disorder 2ndary to stroke that occurred 2 yrs ago ICD-10-CM 2013 Index Sequelae (of) —see also condition   stroke NOS     specified effect NEC I69.398 Seizure(s) (see also Convulsions) R56.9   disorder (see also Epilepsy) G40.909 I69.398 Other sequelae of cerebral infarction Alteration of sensation following cerebral infarction Disturbance of vision following cerebral infarction Use additional code to identify sequelae Codes: G40.909 Epilepsy, unspecified, not intractable, without status epilepticus

Epilepsy Example 3 62 y-o seen for treatment of seizure ICD-10-CM Index Seizure(s) (see also Convulsions) R56.9 R56 Convulsions, not elsewhere classified Excludes1: dissociative convulsions and seizures (F44.5) epileptic convulsions and seizures (G40.-) newborn convulsions and seizures (P90) ICD-10-CM 2013 Tabular R56.9 Unspecified convulsions Convulsion disorder Fit NOS Recurrent convulsions Seizure(s) (convulsive) NOS

Migraine Recurring attacks of moderate to severe throbbing/pulsing pain, often on one side of head Sensitivity to light & sound May have N&V 3 X more common in women Auras = flashing lights/zigzag lines/temporary loss of vision Precursor Migraine Triggers Anxiety Stress Lack of food or sleep Exposure to light Hormonal changes (in women)

Migraine Now believed to have genetic cause Overactive nerve cells responding to low levels of pain TRESK gene Diagnosis (International Headache Society) Type of pain & number of attacks Minimum of 5 attacks Duration of 4-72 hours, if untreated

Migraine and ICD-10-CM Classical migraine = with Aura Intractable Terms that describe intractable migraine include: pharmacoresistant or pharmacologically resistant, treatment resistant, refractory, and poorly controlled. Status migrainosus Migraine attack >72 hours AND Severe Intensity

Migraine Example Pt (Type 2 Diabetic with neuropathy) with weakness of left arm and leg. Brought to Ed where he could speak but not use left arm/leg. Pt recovered and had no neurologic deficits w/in 24 hrs. During encounter he was also treated for an intractable classical migraine. Dx: TIA G45.9 Attack, attacks, transient ischemic (TIA) E11.40 Diabetes, diabetic (mellitus) (sugar), type 2, with, neuropathy G43.119 Migraine, classical – see Migraine, with aura Migraine, with aura, intractable

Other Degenerative Diseases G30-G32 Alzheimer Disease Frontotermoral dementia Senile Degeneration of Brain NEC Degeneration due to Alcohol Abuse

Dementia Significant loss of intellectual abilities, such as memory capacity, severe enough to interfere with social or occupational functioning Criteria for Dx include impairment of attention, orientation, memory, judgment, language, motor and spatial skills, and function Types of Dementia Alzheimer – Most common Vascular Lewy Bodies Frontal Temporal (Pick Disease) Traumatic Other causes AIDS Alcoholism Brain tumors Drug toxicity, Infection Creutzfeldt-Jakob disease Meningitis Syphilis Hypothyroidism

Lewy Body Diseases Lewy bodies – accumulated bits of alpha-synuclein protein – inside nuclei of neurons in brain areas controlling particular aspects of memory/motor control Alpha-synuclein accumulation also linked to Parkinson disease, multiple system atrophy, and several other disorders, - synucleinopathies Dementia with Lewy Bodies (DLB) 1.3 Million in US Parkinson 1 million+ in US (with Dementia Alzheimer 5 million+ in US (4% - Early-onset) = 6th leading cause of death

Lewy Bodies in Brain Lewy body proteins are found Area of brain stem where they deplete neurotransmitter dopamine = Parkinsonian symptoms Lewy body Dementia - abnormal proteins diffuse thru other areas of brain, inclu. cerebral cortex Acetylcholine depleted Disruption of perception, thinking, and behavior (Dementia) http://missinglink.ucsf.edu/lm/ids_104_neurodegenerative/case2/case2micro.htm http://depts.washington.edu/adrcweb/DIMSp11/DIMSp11story1.html

Alzheimer Disease Progressive, degenerative disease of brain Most common form of dementia Commonly affects elderly Early Onset/Younger Onset AD – Before 65 Associated w/development of amyloid plaques in cerebral cortex Characterized by confusion, disorientation, memory failure, speech disturbances, and eventual dementia Unknown cause Named for German psychiatrist Alois Alzheimer (1864-1915) (1906 autopsy)

Risk factors for Alzheimer Disease 65+ - Risk of Alzheimer’s doubles about every five years after age 65 After age 85, risk is nearly 50 % Family hx Parent, sibling or child w/ Alzheimer’s -more likely to develop Risk increases if more family members Heredity Risk genes – Increase likelihood of disease, but do NOT guarantee Risk gene with strongest influence is apolipoprotein E-e4 APOE-e4 may be factor in 20 -25 % of Alzheimer cases Deterministic genes - Directly cause disease, guarantee Genes coding three proteins: amyloid precursor protein (APP), presenilin-1 (PS-1) and presenilin-2 (PS-2) Autosomal dominant Alzheimer’s disease (ADAD) or Familial AD < 5% of all cases 5 more genes related to AD found in 2012

Risk factors for Alzheimer Disease Head trauma May be strong link between serious head injury & risk of Alzheimer’s Esp. when trauma occurs repeatedly or involves LOC Buckling seat belt, wear helmet during sports, and “fall-proof” home Heart-head connection Every heartbeat pumps about 20 -25 % of blood to head Brain cells use at least 20% of food and oxygen in blood High blood pressure cholesterol, Heart disease, Stroke, Diabetes (Insulin Resistance)

Alzheimer Disease Process Amyloid plaques Neurofibrillary tangles Loss of neuron connections Spread to Hippocampus Mild Cognitive Impairment (MCI) Cause: Mix of genetic, environmental, and lifestyle factors

Alzheimer Disease Stages Stage 1: No impairment (normal function) Person does not experience any memory problems. Interview w/ medical professional shows no evidence of symptoms of dementia Stage 2: Very mild cognitive decline (may be normal age-related changes or earliest signs of Alzheimer's disease) May feel having memory lapses — forgetting familiar words or location of everyday objects. No symptoms detected during medical exam or by friends, family or co-workers Stage 3: Mild cognitive decline (early-stage Alzheimer's Dx in some, but not all, individuals w/these symptoms) Friends, family or co-workers begin to notice difficulties. Detailed History by Drs may detect problems in memory/concentration. Common stage 3 difficulties include: Noticeable problems coming up with right word or name Trouble remembering names when meeting new people Noticeably greater difficulty performing tasks in social/work settings Forgetting material that was just read Losing/Misplacing a valuable object Increasing trouble w/planning or organizing

Alzheimer Disease Stages Stage 4: Moderate cognitive decline (Mild or early-stage Alzheimer's disease) Careful medical interview able to detect clear-cut symptoms in several areas Forgetfulness of recent events Impaired ability for challenging mental arithmetic — for example, counting back from 100 by 7s Greater difficulty performing complex tasks - planning dinner for guests, paying bills Forgetfulness about own personal history Becoming moody/withdrawn, especially in socially or mentally challenging situations

Alzheimer Disease Stages Stage 5: Moderately severe cognitive decline (Moderate or mid-stage Alzheimer's disease) Gaps in memory & thinking are noticeable, need help with ADLs Unable to recall own address/telephone number or high school/college Become confused about place or what day it is Trouble w/ less challenging mental arithmetic; such as counting backward from 40 by subtracting 4s or from 20 by 2s Need help choosing proper clothing for season/occasion Still remember significant details about themselves and family Require no assistance with eating or using toilet

Alzheimer Disease Stages Stage 6: Severe cognitive decline (Moderately severe or mid-stage Alzheimer's disease) Memory continues to worsen, personality changes may occur & individuals need extensive help w/ADLs. Individuals may Lose awareness of recent experiences as well as of surroundings Remember own name but have difficulty w/personal history Distinguish familiar & unfamiliar faces but trouble remembering name of spouse Need help to dress properly Experience major changes in sleep — sleeping in day & restless at night Need help handling toileting (i.e., flushing toilet, wiping or disposing of tissue) Increasingly frequent trouble controlling bladder or bowels Major personality/behavioral changes, inclu. suspiciousness & delusions (believing caregiver is impostor) or compulsive, repetitive behavior Tend to wander or become lost

Alzheimer Disease Stages Stage 7: Very severe cognitive decline (Severe or late-stage Alzheimer's disease) Individuals lose ability to respond to environment, to converse and to control movement. May still say words or phrases. Need help with much of daily personal care, including eating or using toilet May also lose ability to smile, to sit w/o support & to hold head up Reflexes become abnormal. Muscles grow rigid. Swallowing impaired. http://www.alz.org/alzheimers_disease_stages_of_alzheimers.asp

Alzheimer Example 52-y-o having increased dementia and forgetfulness. Wanders off from his home & forgets where he is & what he is doing. Dx: Dementia due to Early-onset Alzheimer G30 Alzheimer's disease Alzheimer's dementia senile and presenile forms Use additional code to identify: delirium, if applicable (F05) dementia with behavioral disturbance (F02.81) dementia without behavioral disturbance (F02.80) G30.0 Alzheimer's disease with early onset F02 Dementia in other diseases classified elsewhere Code first underlying physiological condition, such as:Alzheimer's (G30.-) F02.81 Dementia in other diseases classified elsewhere with behavioral disturbance Dementia in other diseases classified elsewhere with aggressive behavior Dementia in other diseases classified elsewhere with combative behavior Dementia in other diseases classified elsewhere with violent behavior Use additional code, if applicable, to identify wandering in dementia in conditions classified elsewhere (Z91.83)

Degeneration due to Alcohol Abuse 20 Million alcoholics in US 50% have mild to severe neuropsychological difficulties Effect depends on multiple variables Alcohol–induced persisting amnesic disorder Amount of alcohol consumed Age began drinking Duration of drinking Pt’s age, level of education, gender, genetic background, and family hx of alcoholism Neuropsychiatric risk factors Alcohol exposure before birth General health status Comorbiditieso

http://pubs.niaaa.nih.gov/publications/arh27-2/125-133.htm

625 Gallons vs. 20 Gallons 1 oz of alcohol = 1 drink 128 oz/gallon X 625 g = 80,000 drinks 128 oz/gallon X 20 g = 2,560 drinks 40 years (57-40 = 17 yo) = 480 months 80,000 = 167/mo = 42/wk = 6/day 480 2,560 480 = 5/mo

Degeneration due to Alcohol Abuse, Comorbidities Medical conditions Malnutrition Liver and Cardiovascular diseases Neurological conditions Head injury, Encephalopathies Fetal alcohol syndrome Psychiatric conditions Depression Anxiety PTSD, Schizophrenia Other drug use

Wernicke-Korsakoff syndrome Neuropsychiatric disorder caused by thiamine deficiency, most often due to alcohol abuse Combines features of Wernicke's encephalopathy and Korsakoff's syndrome Korsakoff's syndrome Syndrome of anterograde & retrograde amnesia wconfabulation associated w/alcoholic or nonalcoholic polyneuritis Wernick encephalopathy Common condition in pts w/long-term alcoholism, resulting largely from thiamin deficiency and characterized by disturbances in ocular motility, pupillary alterations, nystagmus, and ataxia with tremors AKA superior hemorrhagic polioencephalitis, Wernicke disease

Parkinson with Dementia vs. Dementia with Lewy Bodies Dementia symptoms consistent with DLB develop first REM sleep disorder is more common Hallucinations, delusions, and misidentification of familiar people Both dementia symptoms & movement symptoms present at diagnosis Movement symptoms develop w/in year after DLB Dx Disruption of ANS Blood pressure drop on standing, dizziness, falls & urinary incontinence Parkinson's disease dementia Movement symptoms first Dementia symptoms don't appear until year + later

Vascular Dementia (ICD-10-CM Ch 5) Due to cerebrovascular disease, usually with stepwise deterioration Series of small strokes Patchy distribution of neurologic deficits affecting some functions and not others Symptoms include confusion, problems with recent memory, wandering or getting lost in familiar places, bladder or bowel incontinence, emotional problems difficulty following instructions, and problems handling money. Change is noticeable only as series of small steps Over time, more small blood vessels in brain blocked, so more noticeable gradual mental decline. Begins between 60 and 75 Affects men more than women AKA multi-infarct dementia

Demyelinating Diseases G35-G37 Any condition causing damage to protective covering (myelin sheath) surrounding nerve fibers in brain and spinal cord Nerve impulses slow or even stop, causing neurological problems

Demyelination Disease S&S Vision or Hearing loss Headache Seizures Muscle spasms and weakness Loss of coordination Paralysis Loss of sensation

Multiple sclerosis Most common Demyelination Disease AKA – Disseminated sclerosis Thought to be an autoimmune disease Combination of factors Infection, Genes, etc. http://en.wikipedia.org/wiki/File:Symptoms_of_multiple_sclerosis.svg

MS Risk Factors Age - can occur at any age, but most commonly 20s – 40s Gender - Women 2 X more likely Family hx - 1 parent/sibling = 1-3% chance General Population = 0.10% Identical twin has 30% chance of getting IF twin has MS Certain infections - Variety of viruses, such as Epstein-Barr virus and others, appear to be associated with multiple sclerosis. Ethnicity - White people with North European heritage = highest risk AAsian, African or Native American descent = Geographic regions - Europe, southern Canada/northern US, New Zealand & southeastern Australia Child moving from high-risk area to a low-risk area, or vice versa = acquires risk level of new home. Move after puberty, young adult usually retains risk level of first home Other autoimmune diseases - Thyroid disease, type 1 diabetes or inflammatory bowel disease

Other Demyelination Diseases Optic neuritis w/demyelination Inflammation or demyelination of optic nerve AKA retrobulbar neuritis ~ 66% of people with MS have at least one episode of optic neuritis Frequently first symptom of MS One eye at a time, may be pain also Usually clears in 4-12 weeks

Diffuse sclerosis of CNS AKA Periaxial encephalitis, Schilder disease Affects children Dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy

Cerebral Palsy and Other Paralytic Syndromes G80-G83 Many Classification Systems for Cerebral Palsy ICD-10-CM uses terms Spastic Athetoid Quadraplegic Diplegic

Cerebral Palsy Group of nonprogressive disorders of movement and posture AKA Static encephalopathy Caused by abnormal development of, or damage to, motor control centers of brain Events before, during, or after birth 5-10% of CP attributed to birth trauma Other possible causes include Abnormal development of brain Prenatal factors directly /indirectly damaging neurons Preemature birth – 5 X risk; Mulitiple Brain injuries in first few years Most cases are Spastic CP or mixed CP 10,000 new cases in US every year

Cerebral Palsy Risk Factors Maternal health German measles (rubella), viral infection prevented w/ vaccine Chickenpox (varicella), Viral infection prevented with vaccine Cytomegalovirus, very common virus causes flu-like symptoms Birth defects possible if mother has first active infection during pregnancy Toxoplasmosis, parasitic infection in soil and feces of infected cats Syphilis Exposure to toxins, such as methyl mercury Other conditions Thyroid problems, mental retardation or seizures

Cerebral Palsy Risk Factors Infant illness Bacterial meningitis Viral encephalitis Severe or untreated jaundice Other factors of pregnancy and birth Premature birth < 37 weeks = higher risk of cerebral palsy. <28 weeks = 50% chance Low birth weight < 5.5 pounds (2.5 kilograms) = higher risk , which increases as birth weight decreases Breech births Multiple babies. Risk rises number of babies sharing uterus 1+ babies die risk that survivors may have cerebral palsy increases Low Apgar Score at birth

Spastic cerebral palsy Most common manifestation- 70- 80% Hypertonia, or muscle tightness Can affect 1 side of body or just legs Quadriplegia = all four limbs w/spasticity

Athetoid cerebral palsy ~ 25 % of CP AKA dyskinetic cerebral palsy Manifests as slow, involuntary muscle movement accompanied by mixed muscle tone Some muscles have too high tone, while others too low

Ataxic CP Least common type of CP 5 -10 % Characterized by poor muscle tone and difficulty coordinating movements in arms, legs and torso (Cerebellum)

Mixed CP 10 % of CP Several different types of CP in one individual Most common combination (~ 10 percent of all mixed CP cases) Spastic & athetoid movements together All other combinations are also possible, but rarer

Other Disorders of Brain G89-G99

Hydrocephalus Excessive accumulation of fluid in brain Water on the Brain - actually CSF Ventricles abnormally widen – pressure on tissues Blockage of normal CSF flow or absorption Congenital OR Acquired Communicating OR Non-communicating Hydrocephalus ex-vacuo Compensatory replacement by CSF of volume of tissue lost in brain atrophy NPH Normal Pressure Hydrocephalus http://php.med.unsw.edu.au/embryology/index.php?title=File:Hydrocephalus.jpg

Hydrocephalus Dilatation of cerebral ventricles, usually occurring 2ndary to obstruction of CSF pathways Accumulation of CSF w/in skull Typically enlargement of head, prominence of forehead, brain atrophy, mental deterioration, and convulsions Communicating Free access of fluid between ventricles brain and spinal canal Non-communicating (AKA Obstructive) Due to obstruction of CSF flow within brain ventricles or through their exit foramina

NPH CSF balance disrupted S&S May cause normal pressure hydrocephalus Ventriculmegaly Most often in older people Idiopathic OR 2ndary May be result of traumatic fall or injury or illness Can sometimes be reversed (Shunt) S&S Gait and balance problems Urinary incontinence Dementia http://en.wikipedia.org/wiki/File:Hydrocephalus.jpg

Brain Neoplasms

Neoplasms Brain tumors more common than spinal tumors B9 or malignant Almost ½ of brain tumors are B9 Can recur, be fatal Brain common site for 2ndary metastatic brain tumors (from lung, breast ca, etc.)

Gliomas & Other Brain Neoplasms Most common type of nervous system tumors Connective tissue differentiates Astrocytoma Brain stem glioma Ependymoma Oligodendroglioma Medullablastoma Meningioma Neuroma

Astrocytoma Most common glioma ~1/2 of all primary brain & spinal cord tumors Most often in cerebrum Classification Low grade astrocytomas (10%) Anaplastic astrocytomas Glioblastomas Anaplastic astrocytoma Before and After Surgery, Radiation, and Chemo http://commons.wikimedia.org/wiki/File:Anaplastic_astrocytoma.jpg

Brain stem glioma Low grade to high grade Most often in children 3- 10 y-o (75%) Can occur in adults http://www.mypacs.net/cases/BRAIN-STEM-GLIOMA-682746.html

Ependymoma Rare type of glioma (85% B9) Thought to develop from precursor cells to ependymal cells lining ventricles and central canal of spinal cord 3rd most common form of pediatric brain tumors 3-6 % CNS tumors in adults 5% of gliomas in adults, mostly in spine Slightly higher incidence rate in Males & Whites Usually arise from floor of 4th ventricle Can cause Hydrocephalus http://commons.wikimedia.org/wiki/File:Ependymom_sag_FLAIR.jpg

Oligodendroglioma Develops from glial cells (2% of brain tumors) Occurs frequently in frontal/temporal lobes Can be classified as low grade or high grade Common in males and females 20-49 y-o More common in men than women Can occur in children Low to High Grade B9  Malignant CT http://commons.wikimedia.org/wiki/File:Oligodendroglioma_002.jpg

Medulloblastoma Most common at midline of cerebellum & roof of 4th ventricle 25% of all pediatric brain cancers Presents w/ N&V, headache, ataxia, papilledema, nystagmus, irritability, lethargy, cranial nerve palsy, dizziness, altered vision If cells get into CSF, medulloblastoma can spread to other areas of CNS http://www.scielo.br/scielo.php?pid=S0004-282X2003000200008&script=sci_arttext

Meningioma WHO classification B9, slow-growing tumor of meninges, usually next to dura mater May invade skull or cause hyperostosis Often causes increased intracranial pressure Most common in Adults Usually vascular After radiation 2 X more common in women WHO classification Grade I: Benign Meningioma Grade II: Atypical Meningioma Grade III: Malignant (Anaplastic) Meningioma http://upload.wikimedia.org/wikipedia/commons/f/fb/Contrast_enhanced_meningioma.jpg Contrast-enhanced CT of Brain

Neuroma General term for any neoplasm derived from cells of nervous system Acoustic neuroma AKA Vestibular schwannoma Slow-growing B9 tumor of nerve connecting ear to brain (VIII Cranial) Can damage several important nerves as enlarges S&S usually begin in 30s http://en.wikipedia.org/wiki/Vestibular_schwannoma

Brain Traumas

TBI (Traumatic Brain Injury) Disruption of normal brain function Caused by bump, blow or jolt to head or penetrating head injury 1.7+ million every year in US 75% mild (concussion) ~18% of all TBI-related ED visits involved children aged 0 - 4 years ~22% of all TBI-related hospitalizations involved adults 75 years + Males more often diagnosed with TBI (59%).

TBI Causes http://www.cdc.gov/TraumaticBrainInjury/causes.html

Falls Leading cause of TBI in US 50% of TBIs among children 0 - 14 years 61% of all TBIs among adults 65 years + http://en.wikipedia.org/wiki/File:Construction_workers_not_wearing_fall_protection_equipment.jpg

MVAs In all age groups, MVAs & traffic-related incidents 2nd leading cause of TBI (17.3%) Largest percentage of TBI-related deaths (31.8%) 2.3 million+ adult drivers/passengers treated in EDs due to MVAs in 2009 Lifetime costs of crash-related deaths/injuries for drivers/passengers = $70 billion in 2005

Head Trauma Hematoma - collection of blood outside of blood vessel Epidural Subdural Intracerebral Concussion Cerebral Contusion Basilar Skull Fx

Epidural Hematoma (AKA Extra-) Trauma, often to temple Middle meningeal artery Accumulation of blood epidural space Outside (Above) Dura Mater Due to Dura’s attachment to skull, small hematomas can cause significant pressure 1- 3% of head injuries 15-20% are fatal http://en.wikipedia.org/wiki/File:Epidural_Hematoma.jpg

Subdural hematoma Collection of blood on brain’s surface Due to ruptured veins Acute Subdural = one of deadliest head traumas Serious head injury Bleeding rapidly puts pressure on brain tissue Chronic Subdural = common in elderly Can be cause by minor injury (Fall) Can be unnoticed for days/weeks Shrinking brain = more space http://en.wikipedia.org/wiki/File:Subduralandherniation.PNG

Intracerebral hematoma Within brain tissue Caused by bleeding from Uncontrolled high blood pressure Aneurysm leak or rupture Trauma Tumor Stroke http://en.wikipedia.org/wiki/File:Intracerebral_heamorrage.jpg

Concussion L, concutere, to shake violently Trauma-induced loss of consciousness, transient or prolonged Due to a blow to head; may be transient amnesia, vertigo, nausea, weak pulse, and rapid/slow respiration http://en.wikipedia.org/wiki/File:Concussion_mechanics.svg

Contusion of Brain Bruise of Brain tissue 20–30% of severe head injuries Cerebral laceration is similar BUT pia-arachnoid membranes are torn over Injury site Contrecoup contusion Blow on one side of head with damage on opposite side by transmitted force http://en.wikipedia.org/wiki/File:Contrecoup.svg

Basilar Skull Fracture Fx involving base of cranium S&S May be asymptomatic Raccoon eyes Battle sign Hemotympanum CSF Rhinorrhea Imaging: Plain skull film may not reveal fx CT or MRI more reliable http://www.nlm.nih.gov/medlineplus/ency/imagepages/3067.htm

Cerebellar Injuries Trauma Disease Common symptom = Ataxia Lack of muscle coordination Speech pattern changes Abnormal gait http://www.fieldsobrietytests.net/horizontalgazenystagmusfieldsobrietytest.html

Medulla Oblongata Injuries Trauma can be fatal when Reflex centers damaged Non-fatal injuries Cranial nerve malfunction Paralysis Loss of sensation Respiratory Irregularities

Post-Head Injury Dementia Most common causes of head injury MVAs (50%) Falls (21%) Assault or GSW (12%) Sports - boxing (dementia pugilistica), other recreational activity (10%) Use of alcohol/substances factor in ~1/2 Children Bicycle accidents Infants Shaken baby syndrome Elderly persons Especially falls TBI in early/midlife = increased risk of dementia in late life Moderate/severe TBIs = 2-4 X increased risk People < 50 y-o Head injury is 3rd most common cause of dementia, after infection and alcoholism

Homework Select at least TWO of the following sites and review the brain pathologies of your choice Complete the Post -Test HOMEWORK

Homework Brain Tour. Alzheimer’s Association. http://www.alz.org/braintour/3_main_parts.asp Brain Cancer. X-plain Module. http://www.nlm.nih.gov/medlineplus/tutorials/braincancer/htm/index.htm Khurana, V. Brain Tumor Education Resource. 2 Tutorials. http://www.brain-surgery.us/brain_tumor.html Hulman, G. Cerebral Ischaemia and Infarction. Pathology mini-tutorial. Nottingham University Hospitals. http://podcast.ulcc.ac.uk/accounts/UniversityofNottingham/mrzgh-ms3/Cerebral_ischaemia_and_infarction___Medium.mp4 Brain Aneurysms. Toronto Brain Vascular Malformation Study Group. 2001. http://brainavm.oci.utoronto.ca/swf/description_full.html

Homework Parkinson’s Disease. X-plain module. http://www.nlm.nih.gov/medlineplus/tutorials/parkinsonsdisease/htm/index.htm Seizures & Epilepsy. X-plain module http://www.nlm.nih.gov/medlineplus/tutorials/seizuresandepilepsy/htm/index.htm mild Traumatic Brain Injury. US DOD AfterDeployment.org http://www.afterdeployment.org/topics-traumatic-brain-injury Traumatic Brain Injury. X-plain Module. http://www.nlm.nih.gov/medlineplus/tutorials/traumaticbraininjury/htm/index.htm Getting It: A Disease... A Vaccine. National Meningitis Association. Video in 3 parts, 25 min. total. http://www.youtube.com/watch?v=_m6RKgrdS8M

Quizzes Brain Quiz. AARP. http://www.aarp.org/health/brain-health/info-04-2013/brain-health-quiz.html Alzheimer's disease and other types of dementia. FunTrivia.com http://www.funtrivia.com/trivia-quiz/SciTech/Alzheimers-disease-and-other-types-of--dementia-277016.html What Do You Know About Alzheimer's Disease? Yale Medical Group. 10 questions. http://www.yalemedicalgroup.org/stw/Page.asp?PageID=STW022215 Howe, M. How Much Do You Know About Epilepsy? 10 questions. http://www.funtrivia.com/trivia-quiz/SciTech/How-Much-Do-You-Know-About-Epilepsy-339804.html

Quizzes Brain Injury Awareness Quiz - HensonFuerst, Raleigh Brain Injury Lawyers. 8 questions http://www.youtube.com/watch?v=7RnRY_-4kqs Brain Attack. FunTrivia.com http://www.funtrivia.com/trivia-quiz/SciTech/Brain-Attack-326081.html Stroke Quiz: Test Your Medical IQ. eMedicineHealth. http://www.emedicinehealth.com/stroke_quiz_iq/quiz.htm You've Probably Never Heard of It. http://www.funtrivia.com/trivia-quiz/SciTech/Youve-Probably-Never-Heard-of-It-351768.html

Quizzes Health Library. Quizzes. Roper St. Francis Healthcare. Take the Huntington's Disease Quiz. University of Rochester Medical Center. 8 questions. http://www.urmc.rochester.edu/encyclopedia/document.aspx?ContentTypeID=40&ContentID=HuntingtonDiseaseNeuQuiz Health Library. Quizzes. Roper St. Francis Healthcare. http://healthlibrary.rsfh.com/InteractiveTools/Quizzes/ Meningitis Quiz: Test Your Infectious Disease IQ. eMedicineHealth. http://www.emedicinehealth.com/meningitis_quiz_iq/quiz.htm

ANSWERS to FIRST Test Motor Myelinated nerve fibers Limbic system C. Exocrine system. D. Limbic system. ANSWERS to FIRST Test Motor Myelinated nerve fibers Limbic system Carries signals from the outer parts of the body to the CNS Cerebrum Gyri Cerebellum Medullar Oblongata 20% Brainstem Pineal Gland 2 Smell Ventricular 12

Questions ? ? ? ilemten@gmail.com Thank You !

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Resources - Stroke Stroke – Rehabilitation. HeartHealthyWomen.org – Joint project of Cardiovascular Research Foundation, Office on Women's Health, DHHS and WomenHeart: the National Coalition for Women with Heart Disease. http://www.hearthealthywomen.org/cardiovascular-disease/stroke/stroke-page-5.html What Are the Effects of a Frontal Lobe Stroke? About. Com Source: Allan Ropper and Robert Brown, Adam's and Victor's Principles of Neurology, 8th Edition McGraw-Hill Companies Inc, United States of America, 2005, pp 417-430. http://stroke.about.com/od/unwantedeffectsofstroke/f/FrontalStroke.htm What is a stroke? Nat’l Heart, Blood, and Lung Institute. NIH. http://www.nhlbi.nih.gov/health/health-topics/topics/stroke/printall-index.html

Resources - TBI Division of Disability and Aging Services. Vermont. Traumatic Brain Injury. 3 tutorials with workbooks and quizzes. http://www.ddas.vermont.gov/ddas-policies/policies-tbi/policies-tbi-default-page Injury Prevention & Control: Traumatic Brain Injury. CDC. http://www.cdc.gov/TraumaticBrainInjury/index.html Wedro, B. Hematoma. MedicineNet. http://www.medicinenet.com/hematoma/article.htm Brain Injury Guide and Resources. Missouri DHSS and MU DH. CEUs. http://braininjuryeducation.org/

Resources Acoustic neuroma. MedlinePlus. http://www.nlm.nih.gov/medlineplus/ency/article/000778.htm Gray, L. ICD-10-CM Diagnosis Coding for Pain Medicine. CodeitRight Insights. 3/5/2013. http://www.codeitrightonline.com/ciri/icd-10-cm-diagnosis-coding-for-pain-medicine.html Hereditary Spastic Paraplegia Information Page. NINDS. NIH. http://www.ninds.nih.gov/disorders/hereditary_spastic_paraplegia/hereditary_spastic_paraplegia.htm Hydrocephalus Fact Sheet. NINDS. NIH. http://www.ninds.nih.gov/disorders/hydrocephalus/detail_hydrocephalus.htm

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