2 DefinitionsDementia: clinical state characterized by loss of function in multiple cognitive domains; diagnostic features include : memory impairment and at least one of the following: aphasia, apraxia, agnosia, disturbances in executive functioning. Cognitive impairments must be severe enough to cause impairment in social and occupational functioning and there must be a decline from from a previously higher level of functioning.Acute confusional state: impairment of cognitive function that is not progressive, but is reversible. The impairment of consciousness varies, often being worse at night. It may be described as a transient organic brain syndrome characterized by concurrent disorders of attention, perception, thinking, memory, psychomotor behaviour and the sleep-waking cycle.Delirium: acute cognitive and behavioral change with attentional problems (analogous to above)Encephalopathy: diffuse brain dysfunction (includes acute confusional state and delirium)Amnestic syndrome: Partial or total loss of memory, usually resulting from shock, psychological disturbance, brain injury, or illness. (cf Bourne Identity)Mental retardation: a disability characterized by significant limitations both in intellectual functioning and in adaptive behavior as expressed in conceptual, social, and practical adaptive skills beginning before age 18.Schizophrenia: any of several psychotic disorders characterized by distortions of reality and disturbances of thought and language and withdrawal from social contact
3 Schizophrenia Two (or more) of the following, each present for a significant portion of time during a 1-month period (or less ifsuccessfully treated):delusionshallucinationsdisorganized speech (e.g., frequent derailment or incoherence)grossly disorganized or catatonic behaviornegative symptoms, i.e., affective flattening, alogia, or avolition. Note: Only one symptom is required if delusions are bizarre or hallucinations consist of a voice keeping up a running commentary on the person's behavior or thoughts, or two or more voices conversing with each other.
4 Man found down BP: 116/68; 104 HR; 99.5 F; 14 RR Opens eyes to voice; grimaces to pain; unable to follow commands; blinks to threat bilaterallyNormal oculocephalics; symm reactive pupils; facial symmetryReduced tone with withdrawal of all extremities to pain
5 Laboratory Findings Na 152, K 4.1, BUN 76, Cr 2.1; Glc 116 AST/ALT: 23/47; INR 1.9Urine tox neg;serum alc 0Head CT: bifrontal hygromas without mass effect; old parietal encephalomalacia; basal ganglia calcificationCXR: old granulomasEEG: diffuse triphasic waves
6 What is needed to work up confusion? Structural imaging:Brain CTBrain MRIInfection/hemorrhage/tumor evaluation:Spinal tapSeizures/Brain death/psychogenic/other:EEGHerpes EncephalitisFocal status epilepticusOther
7 Confusion in the Nursing Home Dementia with superimposed conditionsInfection: UTI, pneumoniaMedication errors/overdoseEnd-stage medical diseases: CHF, renalPoorly managed diabetesStrokeEncephalitis/meningitisSeizure/post-ictal stateOther
9 38 year old man Talking crazy/staggering around No recent ETHO though has a history of chronic liver disease, coagulopathy, hypertension, seizures, pancreatitis and head traumaMedications: ? Phenytoin and nadololExam: disheveled; 96 F; 179/100 BP; HR 112; disoriented to place, season and is confabulating with poor attention and recall; gaze-evoked nystagmus and incomplete right eye abduction on right gaze; absent reflexes and wide-based ataxic gait.
10 Issues Cognitive syndrome: encephalopathy Diagnosis Treatment Where is the pathology
11 50 yo with mental status changes and abnormal eye movements. Subtle bilateral abnormal hyperintense signal in the paraventricular region of the medial thalami seen on diffusion, flair and T2. Possible subtle abnormal signal of periaqueductal gray matter seen on flair and T2.50 yo with mental status changes and abnormal eye movements.
12 Further history revealed alcohol abuse. Alcohol abuse is the most common etiology. Prompt Thiamine administration is essential and actually was given to the patient prior the this MRI. Wernicke encephalopathy is a medical emergency. Prompt recognition of the symptom complex and a high index of suspicion are crucial to ensure early treatment. Early treatment can rapidly reverse the ophthalmoplegia and improve ataxia/dysequilibrium and early mental confusion, as well as prevent development of the amnestic state. In advanced cases, where severe prolonged deficiency has led to permanent structural damage, permanent deficits most often are manifested as the amnestic state and severe ataxia.Reference: emedicine.Contributor: SandersFindings Subtle bilateral abnormal hyperintense signal in the paraventricular region of the medial thalami seen on diffusion, flair and T2. Possible subtle abnormal signal of periaqueductal gray matter seen on flair and T2.Further history revealed alcohol abuse.Diagnosis Wernicke's encephalopathyDiscussion MRI of the brain with contrast: MRI demonstrates acute lesions of Wernicke-Korsakoff syndrome in medial thalamic and periaqueductal regions. This can be a useful diagnostic procedure in patients presenting with suggestive history and stupor or coma, where ataxia and ophthalmoplegia are not detectable.
15 Chronic Alcohol Effects Cerebellar degeneration Vascular risks ICH SDH ThromboticEmbolicSeizuresCognitiveSpinal cord: B12 defNeuropathyMuscular atrophyHeavy drinkers compared with light or non drinkers are:twice as likely to die of heart diseasetwice as likely to die of cancertwelve times as likely to die of cirrhosis of the liverthree times more likely to die in a car accidentsix times more likely to commit suicide
16 60 year old manMaking mistakes; forgetful; unable to complete his report; no longer interestedIrritable and defensive; lost his way homeGuarded/suspiciousInattentive with digit span of 5; ¼ recall & confabulates 2 othersOccasional paraphasiasDifficulty with 3 step command; problem with 3 D cube drawing
18 www.urmc.rochester.edu/ neuroslides/slide098.html This 80-year-old man presented with a gradual decline in functioning. Examination revealed a marked aphasia and poor visual-spatial ability with an MMSE score of 18/30. These T1-weighted axial MR images reveal diffuse cortical atrophy with prominent sulci and enlarged lateral ventricles.
19 Alzheimer's Disease; neurofibrillary tangles and neuritic plaques: This slide from a silver impregnated section of the hippocampus from a severely demented patient shows numerous neuritic plaques and neurons containing neurofibrillary tangles. The neurofibrillary tangles are intraneuronal, while the neuritic plaques are large extracellular collections of abnormal nerve cell processes, degenerating synaptic terminals, glia and amyloid.neuroslides/slide098.html
29 Initial Symptoms Years Later AD DLBD PDD Dementia Dementia Dementia ParkinsonismDementiaDementiaParkinsonismDementiaParkinsonism
30 Vignette 56 year-old with 6 month history of rapidly progressive dementia, myoclonus andincreased tone
31 SPORADIC CJDThere are three investigations which might provide support for a diagnosis of sporadic CJD. These are:The EEGThe CSF estimationThe MR scanTransverse FLAIR MRI showing bilateral anterior basal ganglia high signal
32 This is an EEG tracing showing the characteristic periodic complexes.
33 78 year old woman Confusion; started “talking crazy” and was stubborn Speaks with “meaningless words” and cannot answer yes/no questions accuratelyCan mime but cannot follow commands, name or repeatUnable to cooperate with most of exam
34 Questions What has happened to this woman? The nature of her deficit What mechanism?Is she aware of her deficits?In what settings is anosognosia seen?Would she be able to read aloud, write or comprehension related to reading?Visual fields would show?Discuss the Wernicke-Geschwind model of language and the anatomical localization
36 SP = Superior Parietal Lobule EC = Exner’s Writing Center A = Angular GyrusB =Broca’s AreaT=ParsTriangularisW = Wernicke’s AreaH = Henschen’sMusic Center
37 DefinitionsAphasia: loss of the ability to use or understand language due to a brain lesionMutism: the condition of being unable or unwilling to speakFluency: "Production and/or perception of verbal elements of communication that adhere to the sequence, rhythm, and timing patterns approriate for the communicative context and expectations of the speaker and/or listener" (Cross, 1998).Paraphasia: A person with aphasia might use an incorrect word or unrecognizable word in place of the target word. This is a paraphasia. Paraphasias can be classified in 3 types. Phonemic or literal paraphasias are word errors that sound very close to the intended word (e.g., coke for coat). A verbal or semantic paraphasia occurs when a word that is related in meaning to the target word is substituted (e.g., plum for peach). The third type of paraphasia is a neologism - an invented word that is not recognizable as a word in the speaker's language.Dysarthria: impaired articulation due to impairment in peripheral nerves or in speech musculatureDysprosody: loss of or deficit in the comprehension or production of nonverbal aspects of language that convey attitudinal, emotional, and similar information to the listener.Apraxia: loss of the ability to produce purposeful, skilled movements as the result of brain damage
38 Aphasias Fluency Comprehension Naming Repetition Global - - - - BrocaWernickeConductionTranscortical-M /- +Transcortical-SAnomicBroca's aphasia, also called motor aphasia, results from damage to the front portion or frontal lobe of the language-dominant area of the brain. Individuals with Broca's aphasia may be completely unable to use speech (mutism) or may be able to use single-word statements or even full sentences, though these sentences may require a great deal of effort to construct. Small words, such as conjunctions (and, or, but) and articles (the, an, a), may be omitted, leading to a "telegraph" quality in their speech. Hearing comprehension is usually not affected, so they are able to understand other people's speech and conversation and can follow commands. Often, they may experience weakness on the right side of their bodies, which can make it difficult to write. Reading ability is impaired, and they may have difficulty finding the right word when speaking. Individuals with Broca's aphasia may become frustrated and depressed because they are aware of their language difficulties.Wernicke's aphasia is caused by damage to the side portion or temporal lobe of the language-dominant area of the brain. Individuals with Wernicke's aphasia speak in long, uninterrupted sentences; however, the words used are frequently unnecessary or even made-up. They have a great deal of difficulty understanding other people's speech, sometimes to the point of being unable to understand spoken language at all. Reading ability is diminished, and although writing ability is retained, what is written may be abnormal. No physical symptoms, such as the right-sided weakness seen with Broca's aphasia, are typically observed. Also, in contrast to Broca's aphasia, individuals with Wernicke's aphasia are not aware of their language errors.Global aphasia is caused by widespread damage to the language areas of the left hemisphere. As a result, all basic language functions are affected, but some areas may be more affected than others. For example, an individual may have difficulty speaking but may be able to write well. The individual may experience weakness and loss of feeling on the right side of their body.Conduction aphasia, also called associative aphasia, is rather uncommon. Individuals with conduction aphasia are unable to repeat words, sentences, and phrases. Speech is fairly unbroken, although individuals may frequently correct themselves and words may be skipped or repeated. Although able to understand spoken language, it may also be difficult for the individual with conduction aphasia to find the right word to describe a person or object. The impact of this condition on reading and writing ability varies. As with other types of aphasia, right-sided weakness or sensory loss may be present.Anomic or nominal aphasia primarily influences an individual's ability to find the right name for a person or object. As a result, an object may be described rather than named. Hearing comprehension, repetition, reading, and writing are not affected, other than by this inability to find the right name. Speech is fluent, except for pauses as the individual tries to recall the right name. Physical symptoms are variable, and some individuals have no symptoms of one-sided weakness or sensory loss.Transcortical aphasia is caused by damage to the language areas of the left hemisphere outside the primary language areas. There are three types of aphasia: transcortical motor aphasia, transcortical sensory aphasia, and mixed transcortical aphasia. All of the transcortical aphasias are distinguished from other types by the individual's ability to repeat words, phrases, or sentences. Other language functions may also be impaired to varying degrees, depending on the extent and particular location of brain damage.
39 73 year old womanSudden onset headache, dizziness with vomiting; unsteadiness of gait and poor coordination of the right armWhat neurological conditions cause sudden, severe headache?What is the localizing value of dizziness, gait instability, and difficulty controlling the RUE?
40 Time PassesPatient is no long able to speak clearly; can open eyes and gruntThen in ER:BP 185/105; afebrile; no nuchal rigidityExtensor posturing with stimulationNo response to voice and no spontaneous limb movementsPupils reactiveEyes deviate to left with cold water in left ear without nystagmus; no response when done to the right ear2 callsTestSpecialist
41 Questions What other parts of the exam is needed Eye movements? Caloric testing results inNormal awake patientComatose patient with intact brainstemBrain-dead patientCharacterize and localize patient’s limb movementsWhat is the diagnosisWhat phone calls were madeWhat is the prognosis
42 Definitions & Underlying Structures ComaPersistent vegetative stateLocked-in syndromeBrain death
51 Management How does increased intracranial pressure (ICP) cause coma? What are the treatments for increased ICP and how do they work?MannitolUreaHyperventilationElevate head of bedSteroids (for vasogenic only)
53 LEVELS OF CONSCIOUSNESS: Alert normal awake and responsive stateLethargic easily aroused with mild stim. Can maintain arousal.Somnolent easily aroused by voice or touch; awakens and follows commands; req stim to maintain arousal Obtunded/Stuporous arousable only with repeated and painful stim; verbal output is unintelligible or nil; some purposeful movement to noxious stimComatose no arousal despite vigorous stim, no purposeful movement- only posturing, brainstem reflexes often absent
55 PUPILS: CN II afferent, CN III efferent PUPILS: CN II afferent, CN III efferent. Tests level of the midbrain as well as autonomic integrity. Some patterns:Hypothalamus: Horner’s (miosis, ptosis, and anhydrosis)Midbrain: midpositoin, fixedPeripheral III: usually unilateral, more dilated, fixedPons: pin point pupilsMedulla (lat): Horner’s- preserved response to lightMetabolic: in general met derangements do not affect pupils. The major exceptions are sympathomimetics and anti-cholinergics which dilate, and opiates which cause pin point pupils.
56 Other Cranial Nerves CORNEALS: V afferent, VII efferent. -pons OCULOCEPHALIC: requires levels intact from III- VIIIGAG: IX, X -medulla
57 MotorCheck for asymmetric response as well as movement that localizes to pain, withdraws from pain, or represents posturing.Posturing: Decorticate: extension LE, flexion at elbows/wrists Better prognosis than decerebrate Often without concomitant loss neuro-optho reflexes Usually lesion is above the midbrainDecerebrate: extension LE, extension/pronation/adduction UE Often with neuro-ophtho changes Most commonly lesion at level of midbrain or di- encephalon
58 V2 incomprehensible sounds V1 nil EYE E4 spontaneous opening Glasgow Coma Scale VERBAL V5 oriented V4 confused V3 inappropriate words V2 incomprehensible sounds V1 nil EYE E4 spontaneous opening E3 opens eyes to speech E2 opens eyes to noxious stim E1 nil MOTOR M6 obeys motor requests M5 localizes to noxious stim M4 withdrawal from noxious stim M3 abnormal flexion response (decorticate posturing) M2 abnormal extension (decerebrate posturing) M1 nil