1. LMCC Preparation Back to Basics Neurology Dr. C.R. Skinner 17 April 2008

2. Major Topics Neurology Made Simple Review Headache Trauma Infections Cerebrovascular Disease Demyelination Seizures Degenerative Diseases Sleep Disorders

3. Neurology Made Simple Questions Is the Problem Neurological?Is the Problem Neurological? If So, Where Is It in the NervousIf So, Where Is It in the Nervous System ? System ? What Is the Most Likely Cause ?What Is the Most Likely Cause ? Is This Problem Serious Enough to Require Urgent Referral ?Is This Problem Serious Enough to Require Urgent Referral ? How to Stabilize the Patient During TransportHow to Stabilize the Patient During Transport

4. NEUROLOGY MADE SIMPLE IS THE PROBLEM NEUROLOGICAL? 4Are there hard organic features ? 4Is the behaviour bizarre ? 4Is there a history of seizures, drug addiction or of psychiatric illness ? 4Do the signs and symptoms make sense ?

5. NEUROLOGY MADE SIMPLE INTRODUCTION 4NEUROLOGICAL PROBLEM 4WHERE IS THE LESION 4WHAT IS THE CAUSE 4INVESTIGATION 4TREATMENT 4PROGNOSIS

6. Localization Matrix

7. NEUROLOGY MADE SIMPLE INTRODUCTION 4NEUROLOGICAL PROBLEM 4WHERE IS THE LESION 4WHAT IS THE CAUSE 4INVESTIGATION 4TREATMENT 4PROGNOSIS

8. Etiology Matrix

9. NEUROLOGY NEUROLOGY MADE SIMPLE INVESTIGATIONS 4History 4Physical 4According to presumed localization and etiology 4Metabolic systemic CSF 4Structural 4Neurophysiological

10. Time Sequence When was the person last neurologically normal? What was the speed of onset of the symptoms? What was the state of the patient’s health in the last few days, weeks, months? What medications is the patient taking and has there been any recent changes? Are there any significant other medical or surgical problems?

11. Headache Loss Of Consciousness Weakness Difficulty With Vision, Hearing, Taste NumbnessWeaknessDizziness Lightheadedness, Vertigo, Off balance Fatigue Loss Of Balance Loss Of Coordination Difficulty Swallowing, Chewing Urinary And Stool Incontinence Sexual Dysfunction Sleep Difficulties HISTORICAL ESSENTIAL HISTORICAL FEATURES HISTORY OF PRESENT ILLNESS

12. Etiology Matrix

14. SIMPLE NEUROLOGY MADE SIMPLE LOCALIZATION ANALYSIS 4Muscle 4Neuromuscular junction 4Peripheral nerve 4Spinal cord 4Brain stem 4Thalamus or basal ganglia 4Cortex 4Cerebellum

15. Localization Matrix

16. Muscle

17. Neuromuscular Junction

18. Peripheral Nerves

25. Lateral Medullary Syndrome IpsilateralIpsilateral –Pain numbness, impaired sensation over half of face –Ataxia of limbs with falling towards side of lesion –Vertigo, nausea, vomiting –Horner’s syndrome ContralateralContralateral –Impaired pain and temperature sensation over half of the body and some of the face

26. Lateral Pontine Syndrome IpsilateralIpsilateral –Horizontal, vertical nystagmus vertigo, nausea, vomiting, oscillopsia –Facial paralysis –Paralysis of conjugate gaze to side of lesion –Deafness, tinnitus –Ataxia –Impaired sensation over face ContralateralContralateral –Impaired pain and thermal sense over half of body

27. Ventral Midbrain Syndrome Weber’s Syndrome IpsilateralIpsilateral Diplopia, dilated pupilDiplopia, dilated pupil AtaxiaAtaxia ContralateralContralateral Hemiplegia, mainly upper limb and faceHemiplegia, mainly upper limb and face

28. CORRELATIVE FACTORS CAPSULE, THALAMUS AND BASAL GANGLIA Hemi-sensory or motor signs Hemi-sensory or motor signs Sensory signs of primary modalities Sensory signs of primary modalities Sensory involvement of the trunk Sensory involvement of the trunk Lack of cortical signs Lack of cortical signs Uniform motor signs in arm, leg and face Uniform motor signs in arm, leg and face Hypertension risk factor Hypertension risk factor

29. CORRELATIVE FACTORS CORTICAL Aphasia and right sided weakness fluent, non - fluent, paraphasia Aphasia and right sided weakness fluent, non - fluent, paraphasia Weakness - arm and face more than leg Weakness - arm and face more than leg Visual field defects Visual field defects Cortical sensory disturbance inattention left-right acalculia agnosia apraxia Cortical sensory disturbance inattention left-right acalculia agnosia apraxia

30. Cerebellum Basal Ganglia Modulating structuresModulating structures Rule out other systems firstRule out other systems first Ipsilateral Rule for pure cerebellar lesionsIpsilateral Rule for pure cerebellar lesions

31. FINAL CHECKLIST THINGS NOT TO MISS 4MUSCLE POLYMYOSITIS, POLYMYALGIA RHEUMATICA 4NEUROMUSCULAR MYASTHENIA 4PERIPHERAL NERVE GUILLAIN - BARRE SYNDROME 4SPINAL CORD ACUTE SPINAL CORD COMPRESSION

32. CHECKLIST FINAL CHECKLIST THINGS NOT TO MISS 4BRAIN STEM 4STROKE, MULTIPLE SCLEROSIS 4MENINGITIS LISERIA, TB 4GUILLAIN - BARRE - FISHER VARIANT 4TUMORS

33. FINAL CHECKLIST THINGS NOT TO MISS 4CORTEX 4STROKE 4HERPES ENCEPHALTIS 4SEIZURES 4TUMORS 4SUBARACHNOID HEMORRHAGE

34. Circulation du LCR Circulation of CSF

35. This 20 year old man presents with a three day history of abnormal behaviour consisting of hallucinations, delusions of grandeur and memory loss for recent events. He had been " up north " fishing just prior to the onset of these symptoms. Case

36. Physical ExamPhysical Exam Fever 38.0 CFever 38.0 C InattentiveInattentive Poor short term memoryPoor short term memory Left upper quadrantopsiaLeft upper quadrantopsia Hyperflexia Left upper and lower limbHyperflexia Left upper and lower limb Case

37. Case 1 Where is the lesion?Where is the lesion? – why? What is the cause?What is the cause? What are the immediate treatment priorities?What are the immediate treatment priorities?

40. Herpes Simplex Encephalitis Any time of year, any age, any sex Selective infection of temporal lobes New onset seizures or behaviour disturbance Treat if you suspect - Acyclovir 30 mgm/kg-day

41. Herpes Simplex Treatment Acyclovir IVAcyclovir IV Management of ICP (max at 8 – 10 Days)Management of ICP (max at 8 – 10 Days) –Head up –Hyperventilation –Mannitol –Hypertonic Saline Seizure treatmentSeizure treatment

42. CASE 2 This 24 year old soldier was doing his early morning run with his regimental company. He developed an acute severe headache which caused him to stop and fall to the ground. On examination, he was alert, oriented, moving all four limbs with a normal neurological examination. Where is the lesion ?

43. CT Scan without contrast

44. Subarachoid Hemorrhage CT Scan Subarachnoid Blood

45. Subarachnoid Hemorrhage Worse headache of lifeWorse headache of life Sudden onset, often with activitySudden onset, often with activity Signs of meningeal irritationSigns of meningeal irritation –Kernig, Brudzinski Focal signsFocal signs Signs of comaSigns of coma Positive CT scanPositive CT scan Positive LPPositive LP

46. Subarachnoid Hemorrhage Blood in subarachnoid space Require urgent referral for angiogram Use acetaminophen not ASA for headache

47. Subarachnoid Hemorrhage Investigations CT Scan - 90 - 95% sensitive LP - nearly 100% sensitive –rbc in CSF –xanthochromic in CSF after 12 – 18 hours Angiogram Treatment –surgical clipping –coiling

48. Subarachnoid Hemorrhage Treatment Clipping Coiling

49. Giant Aneurysm

50. GDC Coil

51. Headache in the Emergency Room 1. Distinguish ominous from benign headache 2. Treat effectively the benign headaches

52. Assessment Approach AirwayBreathingCirculationDrugsEvaluation Assess, secure Not a problem unless obtunded Assess, assist Not a problem unless obtunded O2 not necessary IV line with crystalloid to restore volume No drugs until diagnosed, unless required to stablize circulation In particular, no analgesics until diagnosed Rapid neurological assessment Investigations as necessary

53. The Spectrum of Ominous Headaches Subarachnoid hemorrhageSubarachnoid hemorrhage meningitismeningitis increased intracranial pressureincreased intracranial pressure

54. Danger Signals the worse headache everthe worse headache ever onset with exertiononset with exertion decreased level of consciousnessdecreased level of consciousness meningeal irritationmeningeal irritation abnormal physical signs (including fever)abnormal physical signs (including fever) worseningworsening

55. All Clear Signals previous identical headachesprevious identical headaches patient bright and alertpatient bright and alert neck suppleneck supple –Kernig, Brudzinski’s signs normal examinationnormal examination improving without analgesicsimproving without analgesics

56. CT Scan detects most conditions causing increased ICPdetects most conditions causing increased ICP misses 10 - 15 % of subarachnoid hemorrhagemisses 10 - 15 % of subarachnoid hemorrhage misses nearly all cases of meningitismisses nearly all cases of meningitis

57. Modified HIS Diagnostic Criteria for Migraine multiple previous attacksmultiple previous attacks duration of attacks a few hours to a few daysduration of attacks a few hours to a few days headaches have at least two of:headaches have at least two of: hemicranialhemicranial severesevere pulsatingpulsating worse with activityworse with activity headaches accompanied by at least one of:headaches accompanied by at least one of: nausea and or vomitingnausea and or vomiting aversion to light or noiseaversion to light or noise no evidence of ominous disease in history or examinationno evidence of ominous disease in history or examination

58. Classification of Primary Headaches MigraineMigraine –with aura, without aura –Ophthalmoplegic, retinal Tension HeadacheTension Headache Cluster HeadacheCluster Headache Miscellaneous without structural lesionMiscellaneous without structural lesion

59. Treating Migraine in the ER 1. Restore intravascular volume 2. Identify contraindications 3. Choose appropriate medication

60. Narcotic - Antinauseant Meperidine 75 - 100 mg plusMeperidine 75 - 100 mg plus Prochlorperazine 5-10 mgProchlorperazine 5-10 mg IV slow pushIV slow push

61. DHE - Antinauseant Metoclopramide 10 mg IV, followed in 10 minutes byMetoclopramide 10 mg IV, followed in 10 minutes by DHE 0.5 - 1.0 mg IV by slow pushDHE 0.5 - 1.0 mg IV by slow push

62. Chlorpromazine Ensure patient is normovolemicEnsure patient is normovolemic –250 - 500 cc crystalloid Prepare CPZ for injectionPrepare CPZ for injection –25 mg (1ml) CPZ diluted to 5 ml by adding 4 ml of crystalloid –each ml contains 5 mg CPZ Inject into IV tubing 5 mg CPZ every 10 - 15 minutes, stopping whenInject into IV tubing 5 mg CPZ every 10 - 15 minutes, stopping when –improvement clearly occurring, or –25 mg given –Watch for hypotension and sedation

63. Nasal Sprays DHE DHE Sumatriptan Sumatriptan

64. Sumatriptan 1 mg SC if: no ergot or DHE in past 24 hoursno ergot or DHE in past 24 hours no contraindicationno contraindication

65. Patient Instructions: Guidelines Do not drive a car or operate machineryDo not drive a car or operate machinery Do not drink alcohol or take tranquillizers, antihistamines, or other drugs that affect the CNSDo not drink alcohol or take tranquillizers, antihistamines, or other drugs that affect the CNS Store in child-resistant containersStore in child-resistant containers Neurological followup if frequent or incapacitatingNeurological followup if frequent or incapacitating

66. Cluster Headache 1/10 as common as migraine1/10 as common as migraine Not genetically determinedNot genetically determined M:F 6:1M:F 6:1 Later OnsetLater Onset RhythmicityRhythmicity Severe unilateral orbital, supraorbital and/or temporal painSevere unilateral orbital, supraorbital and/or temporal pain

67. Cluster Headache IpspilateralIpspilateral –conjunctival injection –nasal congestion –forehead and facial swelling –miosis –ptosis –eyelid edema Every 2 - 8 times per dayEvery 2 - 8 times per day

68. Cluster Headache TriggerTrigger –alcohol PathophysiologyPathophysiology –Role of proximal internal carotid artery –Role of histamine TreatmentTreatment Acute attackAcute attack –ergotamine

69. Cluster Headache ProphylaxisProphylaxis –Sansert –Steroids –Lithium –Calcium channel blockers –Combinations

70. Inflammatory Headaches Most people who think they have sinus headaches usually have migraine or muscle contraction headacheMost people who think they have sinus headaches usually have migraine or muscle contraction headache Diagnosis requires acute sinusitisDiagnosis requires acute sinusitis Nasal congestion, post nasal drip, fever, pain over the involved sinusesNasal congestion, post nasal drip, fever, pain over the involved sinuses Tender on percussionTender on percussion Sinus xray confirmsSinus xray confirms TreatmentTreatment –Antibiotics or drainage

71. Temporal Arteritis Progressively obliterative granulomatous arteritisProgressively obliterative granulomatous arteritis Temporal or occipitalTemporal or occipital Can involve cerebral and ophthalmic arteriesCan involve cerebral and ophthalmic arteries 50% will go blind and have a stroke50% will go blind and have a stroke Greater than > yearsGreater than > years

72. Temporal Arteritis Usually temporal headacheUsually temporal headache Malaise, anorexia, night sweats, myalgia, +/- fever, jaw claudicationMalaise, anorexia, night sweats, myalgia, +/- fever, jaw claudication ESR > 50ESR > 50 DiagnosisDiagnosis –Superficial temporal artery biopsy TreatmentTreatment –Prednisone 60 -100 mgm daily

73. Meningeal Irritiation Meningitis and subarachnoid hemorrhageMeningitis and subarachnoid hemorrhage Occurs due to inflammation and intracranial pain sensitiveOccurs due to inflammation and intracranial pain sensitive structures.structures. Subarachnoid hemorrhage - sudden onset meningitisSubarachnoid hemorrhage - sudden onset meningitis Meningitis - more gradual.Meningitis - more gradual.

74. Meningeal Irritiation Occipital and nuchal pain.Occipital and nuchal pain. Interscapular and low back pain.Interscapular and low back pain. Aggravated by movementsAggravated by movements Photophobia, vomitingPhotophobia, vomiting FeverFever DiagnosisDiagnosis History and physicalHistory and physical CT LPCT LP

75. Headache and Brain tumor Traction on intracranial pain sensitive structures.Traction on intracranial pain sensitive structures. Progressively worsening headache.Progressively worsening headache. Morning headaches.Morning headaches. Worsen in head down position.Worsen in head down position. Worsen with cough and strainingWorsen with cough and straining May be localized (constant).May be localized (constant). Focal neurologic signs.Focal neurologic signs.

76. Benign Headaches Hierarchy of Treatment Acute Treatment –Low tech first –Prevention –Acetaminophen alternate with NSAID –Adequate doses and timing according to body weight –Avoid codeine

77. Hierarchy of Treatment Acetaminophen/NSAIDS Muscle Relaxants/Topiramate/Valproate Narcotics/Steroids/Oxygen Triptans/Chlorpromazine

78. Hierarchy of Prevention Risk Factors – Headache Diary Alcohol/Foods/Sleep deprivation/Meals/Stress management/Repetitive Stress injury Amitriptyline/Beta blockers/pizotyline/Singulair Lithium/DBS Lithium/DBS Valproate/Chlorpromazine

79. Case 22 year old man develops double vision especially when looking up or to either side 22 year old man develops double vision especially when looking up or to either side He has noted some increased fatiguability of his muscles He has noted some increased fatiguability of his muscles EOM shown in video EOM shown in video Exam otherwise normal Exam otherwise normal

81. Case He is then given 10 mgm of IV Tensilon (Edrophonium) He is then given 10 mgm of IV Tensilon (Edrophonium) His extra ocular movements are then reexamined His extra ocular movements are then reexamined

83. Case Where is the lesion? Where is the lesion? What other tests might be used? What other tests might be used? What is the commonest treatment? What is the commonest treatment? What are the long term risks of the disease? What are the long term risks of the disease?

84. Myasthenia Gravis Definition * Autoimmune disease with destruction Of neuromuscular junctions * Symptoms - Progressive fatigibility over time

85. Myasthenia Gravis Symptoms * Progressive fatigibility over time * Double vision * Drooping of eyelid(s) * Difficulty swallowing * Change in voice * Difficulty breathing

86. Myasthenia Gravis Signs * Weakness of eyes movements * Weakness of facial muscles * Weakness of swallowing, cough Or gag * Weakness of limbs * No sensory loss

87. Myasthenia Gravis

88. Myasthenia Gravis Investigations Tensilon Test EMG Anti-acetylcholine receptor antibodies

89. Myasthenia Gravis Treatment Mestinon (pyridostigmine) Steroids IV IgG Plasma exchange Thymectomy

90. CASE This 65 year old man present with acute aphasia and right sided weakness. On examination, he has a right facial droop, weakness of the right arm greater than the leg and global aphasia. Where is the lesion ? What is the etiology ?

91. CT Scans

92. Left ACA and MCA Territory Infarction CT Scans

93. Left Carotid Stenosis Angiogram Pathology

94. Stroke 50,000 new cases per year50,000 new cases per year #3 cause of death#3 cause of death

95. Risk Factors age - doubles every decade after 55 yearsage - doubles every decade after 55 years sex - males 25% greater than femalessex - males 25% greater than females blood pressure - 5 timesblood pressure - 5 times CholesterolCholesterol Sleep apnea – 3 –4 timesSleep apnea – 3 –4 times smoking - 4 timessmoking - 4 times alcohol abuse - 3 timesalcohol abuse - 3 times diabetes - 3 timesdiabetes - 3 times homocysteinhomocystein

96. Definition Transient ischemic attackTransient ischemic attack –Focal cerebral ischemic event resolving within 24 hours Completed StrokeCompleted Stroke –No resolution of symptoms

97. Causes of Stroke 85% infarct -85% infarct - – 60% cerebral atherosclerosis –20% lacunar –15 cardiogenic emboli 15% hemorrhage –15% hemorrhage – –intracerebral –subarachnoid hemorrhage

98. Cerebral Circulation 2 Vertebral Arteries  Basilar 2 Carotid Arteries Circle of Willis

99. CT Angiogram

100. Vascular Distribution

102. Lacune Fibrinoid degeneration of penetrating arteriesFibrinoid degeneration of penetrating arteries Most commonly due to hypertensionMost commonly due to hypertension Involves deep white matter pens and basal gangliaInvolves deep white matter pens and basal ganglia

103. Cardiogenic Emholi NVAF -45%.NVAF -45%. IHD - acute MI -15%IHD - acute MI -15% - ventricular aneurysm -10%- ventricular aneurysm -10% RHD -10%RHD -10% Prosthetic valve -10%Prosthetic valve -10% Other -10%.Other -10%.

104. Stroke Syndromes Carotid Circulation Internal Cerebral Artery HemiplegiaHemiplegia Hemisensory lossHemisensory loss Aphasia (L)Aphasia (L) Neglect (R)Neglect (R)

105. Anterior Cerebral artery Contralateral lower limb paresis and hyperreflexiaContralateral lower limb paresis and hyperreflexia upper limb relatively sparedupper limb relatively spared

106. PCA Infarct

107. Vertebrobasilar Circulation Multiple stroke syndromes depending on vessels in siteMultiple stroke syndromes depending on vessels in site DysarthriaDysarthria AtaxiaAtaxia Bilateral weaknessBilateral weakness Bilateral sensory complaintsBilateral sensory complaints Bilateral visual complaintsBilateral visual complaints

108. Pontine Infarct Locked-In Syndrome

109. Lacunar Infarct Multiple stroke syndromes but in general produces pure motor or pure sensory strokeMultiple stroke syndromes but in general produces pure motor or pure sensory stroke

110. Isolated Symptoms Rarely due to Stroke or TIA Vertigo. dizzinessVertigo. dizziness DiplopiaDiplopia Loss of consciousnessLoss of consciousness ConfusionConfusion

111. Differential Diagnosis Transient Ischemic attackTransient Ischemic attack Focal seizureFocal seizure HypoglycemiaHypoglycemia Carpal tunnel syndromeCarpal tunnel syndrome MigraineMigraine HysteriaHysteria

112. Ddx Vertebrobasilar TIA SyncopeSyncope LabyrinthitisLabyrinthitis Myasthenia gravisMyasthenia gravis Meniere's diseaseMeniere's disease

113. Investigation CT, CTA, CT perfusionCT, CTA, CT perfusion MRI/MR angiogramMRI/MR angiogram CBC. differential and plateletsCBC. differential and platelets INR, PTTINR, PTT Cholesterol, triglycerideCholesterol, triglyceride DopplerDoppler EchocardiogramEchocardiogram

114. Vertebrobasilar Circulation As above but no DopplerAs above but no Doppler TreatmentTreatment Prevention:Prevention: Controlled risk factorsControlled risk factors

115. Carotid Circulation If no severe carotid stenosis or cardiac embolusIf no severe carotid stenosis or cardiac embolus antiplatelet agents. specifically aspirin, Plavix or Ticlid.antiplatelet agents. specifically aspirin, Plavix or Ticlid. Cardiac embolus is treated with CoumadinCardiac embolus is treated with Coumadin Carotid stenosis severe than 70% is treated with carotid endarterectomy or angioplasty and stentingCarotid stenosis severe than 70% is treated with carotid endarterectomy or angioplasty and stenting Risk factor managementRisk factor management

116. Acute Thrombolysis

117. Pre Post

118. Treatment of Acute Stroke NINDS protocol –< 3 hours since onset –< 1/3 of MCA territory –No recent bleeding or surgery –IV r-tpa and/or intraarterial tpa angioplasty, stent

119. Brain Attack Distribution of Hemorrhages

121. Case History 22 yr old male assaulted in a bar at 2400 hrs Had been drinking Loss of consciousness ?? Vomited twice Brought to ED by car at 0100 “Alert and oriented” x3 @ RN

122. Case cont’d Seen by EP at 0230: –ambulatory; not distressed –GCS 15 –amnesia x 5 min before injury –object recall 3/3 –forehead contusion but no signs of open or basilar #

123. Case cont’d What would you do? a) observe overnight b) do CT scan immediately c) do CT scan in a.m. d) discharge home with head injury sheet

124. Case cont’d Discharged home with friends Returned 36 hrs later c/o headache and dizziness Ambulatory; not distressed GCS 15 Neurologically intact CT ordered

125. Skull fracture

126. epidural What would be the symptoms and signs? What would be the treatment?

127. ACUTE EPIDURAL HEMATOMA Note the biconvex hyperdense area (arrows). The blood collection is between the skull and dura. It crosses dural attachments, but not sutures. The etiology is secondary to a lacerated meningeal artery or dural sinus. The Subdural windows may help to discern extra- axial collections such as blood in this case.

128. ACUTE SUBDURAL HEMATOMA WITH SUBFALCINE HERNIATION The arrow heads point to the presence of subfalcine herniation (midline shift). This is secondary to the mass effect caused by the moderate sized acute subdural hematoma (arrow) overlying the right fronto-temporal convexity.

129. Acute on chronic SDH What would be the symptoms and signs? What would be the treatment?

130. RIGHT FRONTAL PETECHIAL HEMORRHAGIC CONTUSION Contusions may be hemorrhagic or nonhemorrhagic. They are part of the spectrum of cranio- cerebral trauma. Although there is no obvious mass effect, and there may not be neurologic deficits solely due to the presence of this particular lesion, there are usually areas of associated brain injury known as diffuse axonal injury (DAI) which may account for more severe neurologic deficits. An MRI will demonstrate more subtle anomalies which cannot be demonstrated on CT scan examination.

131. Subdural hematoma Drowsiness. headacheDrowsiness. headache Evolves over days to weeksEvolves over days to weeks History of head trauma not always presentHistory of head trauma not always present

132. Subdural Hematoma

133. Case 24 year old woman presents with decreased vision in right eye No history of recent febrile illness Exam shows decreased colour vision in right eye and bilateral hyperreflexia Where is the lesion?

134. Multiple Sclerosis MRI

135. Demyelinating Disease Classification Multiple sclerosis.Multiple sclerosis. Diffuse cerebral sclerosis.Diffuse cerebral sclerosis. Post viral or post vaccinial disseminated encephalomyelitis.Post viral or post vaccinial disseminated encephalomyelitis. Necrotizing hemorrhagic encephalomyelitis.Necrotizing hemorrhagic encephalomyelitis. Metabolic toxicMetabolic toxic postanoxic encephalopathypostanoxic encephalopathy CPMCPM

136. Dvsmvelinating Disorders ALDALD MLDMLD Krabbe'sKrabbe's Canavan'sCanavan's Chediak HigashiChediak Higashi Neuraxonal dystrophyNeuraxonal dystrophy Pelazeus MerzbacherPelazeus Merzbacher

137. Multiple Sclerosis Dissemination of lesions in time and spaceDissemination of lesions in time and space MacDonald CriteriaMacDonald Criteria EDSS or Kurtze ScaleEDSS or Kurtze Scale Prevalence 0.1%Prevalence 0.1% Female:male = 3:2Female:male = 3:2 20 to 40 years old (peak age 28 years)20 to 40 years old (peak age 28 years)

138. The Expanded Disability Status Scale (EDSS), or Kurtzke scale, gauges the extent of a person's disability by measuring the level of neurologic impairment. Following is a breakdown of the EDSS: 0 – 1 No disability, minimal signs on one FS* (functional system) 1 – 2 Minimal disability in 1 FS 2 – 3 Moderate disability in 1 FS or mild disability in 3-4 FS, though fully ambulatory 3 – 4 Fully ambulatory without aid, up and about 12 hours/day, despite relatively severe disability; able to walk without aid for 500 meters 4 – 5 Ambulatory without aid for about 200 meters; disability impairs full daily activities 5 – 6 Intermittent or unilateral constant assistance required to walk 100 meters with or without resting 6 – 7 Unable to walk beyond 5 meters even with aid, essentially restricted to wheelchair, wheels self, transfers alone 7 – 8 Essentially restricted to bed or chair or perambulated in wheelchair, but may be out of bed much of day; retains self-care functions, generally effective use of arms 8 – 9 Helpless bed patient, can communicate and eat 9 - 9.5 Unable to communicate effectively or eat/swallow 10 Death due to multiple sclerosis

139. Practical use of MRI in the diagnosis and management of patients with MS

140. Key Features for the Diagnosis of MS Dissemination in time and space of lesions typical of MS Exclusion of other better explanations for the clinical features

141. Lesions in Space Clinical evidence must be an objective sign MRI evidence should meet 3 out of 4 Barkhof criteria (minimum of 2 to 9 lesions depending on use of gadolinium and location of lesions), or 2 lesions and abnormal CSF

142. Lesions in Time Clinical attacks should be: >24 hours duration and separated by > 30 days Consistent with demyelination Not a pseudoattack Requires an objective finding MRI attacks can be: A new gadolinium enhancing lesion or A new T2 lesion Separated by > 3 months from clinical event

143. Paraclinical Tests MRI  Most sensitive and specific Visual evoked potentials  Can be used as objective clinical evidence  Delayed with preserved wave form CSF required for:  Equivocal MRI  PPMS diagnosis  Unusual clinical picture

144. What is a Positive MRI (Barkhof Criteria)? 3 out of 4 of the following: 9 T2 lesions or 1 Gad-enhancing lesion 1 infratentorial lesion 1 juxtacortical lesion 3 periventricular lesion Minimum lesions required 5 (2 if Gad used) Note: 1 spinal cord lesion = 1 brain lesion

145. What is MRI Evidence for Dissemination in Time? At least 3 months after the clinical attack: 1 Gad-enhancing lesion At least 3 months after the last MRI scan: 1 new T2 lesion or 1 Gad-enhancing lesion

146. Definite MS (DMS) Requirements: 2 Clinical attacks 2 objective signs 1 objective sign 0–1 MRI required None ― but recommended (Caution if MRI and CSF are normal) 3 of 4 Barkhof criteria or 2 MRI lesions and abnormal CSF 1 Clinical attack 2 objective signs 1 objective sign 1–3 MRIs required Gd lesion > 3 months after clinical attack or New T2 or Gd lesion > 3 months after 1st MRI 2–3 MRIs required Positive 1st MRI AND Gd lesion > 3 months after clinical attack

147. Treatment Acute AttacksAcute Attacks Steroids.Steroids. – Solumedrol –Prednisone not for optic neuritis ProphylaxisProphylaxis –Beta interferon –Copolymer –Mitoxandrone –Natalizumab SymptomaticSymptomatic SpasticitySpasticity –Baclofen –Dantrium –Benzodiazepines

148. Treatment Paroxysmal DisordersParoxysmal Disorders –Tegretol –Dilantin Bladder DysfunctionBladder Dysfunction –Anticholinergic drugs eg. (Ditropan) –Antibiotic treatment DepressionDepression –Tricyclic antidepressants FatigueFatigue –Amantidine

149. Seizures Seizures originate from the cortex Case

151. Simple Partial Seizures. 1. motor 2. somatosensory or special sensory 3. autonomic 4. psychic

152. Complex Partial Seizures Partial seizure with altered awarenessPartial seizure with altered awareness SP -CPSP -CP CPCP + automatisms.+ automatisms.

153. Partial Seizures - to secondary generalization SP-GTCSP-GTC CP - GTCCP - GTC SP -CP-GTCSP -CP-GTC

154. Generalized Seizures (convulsive and non convulsive) AbsenceAbsence –Typical –Atypical MyoclonicMyoclonic ClonicClonic TonicTonic Tonic-clonicTonic-clonic Atonic.Atonic.

155. History How did the seizure start How did the seizure start –staring –eye deviation –jerking or one limb Patient's description of auraPatient's description of aura Post ictal statePost ictal state Age of onsetAge of onset Family historyFamily history Past historyPast history Alcohol or drug abuseAlcohol or drug abuse

156. Physical Exam Todds paralysisTodds paralysis Eye deviationEye deviation Cranial bruitCranial bruit HyperventilationHyperventilation Skin examinationSkin examination IncontinenceIncontinence

157. Investigation Glucose. BUN, Calcium. SodiumGlucose. BUN, Calcium. Sodium CT, MRICT, MRI EEGEEG

158. Treatment Partial Seizures CarbamazepineCarbamazepine PhenytoinPhenytoin PrimidonePrimidone PhenobarbitalPhenobarbital Valproic AcidValproic Acid

159. Tonic Clonic Tonic Clonic Valproic acidValproic acid CarbamazepineCarbamazepine PhenytoinPhenytoin PhenobarbitalPhenobarbital PrimidonePrimidone

160. Absence Absence ValproateValproate EthosuximideEthosuximide

161. Mvoclonic ValproateValproate ClonazepamClonazepam NitrazepamNitrazepam

162. Add On Meds Gabapentin Lamictal Vigabatrin Topiramate

163. CASE This 23 year gay man has had progressive cognitive impairment in the last 3 months which has been complicated by PCP pneumonia from which he is recovering. This 23 year gay man has had progressive cognitive impairment in the last 3 months which has been complicated by PCP pneumonia from which he is recovering. On examination, he has general mental slowing with some tremor in the left upper limb. On examination, he has general mental slowing with some tremor in the left upper limb. Where is the lesion ? Where is the lesion ?

164. CT Scan

165. Dementia A loss of intellectual ability of sufficient severity to interfere with social or occupational functioning.A loss of intellectual ability of sufficient severity to interfere with social or occupational functioning. Memory impairment.Memory impairment. At least one of:At least one of: –impaired abstract thinking –Impaired judgement –Other disturbances of higher cortical functioning - –aphasia. apraxia, agnosia. constructional difficulty –Personality change

166. Irreversible Alzheimer’s Disease, Frontotemporal Dementia (tauopathies)Alzheimer’s Disease, Frontotemporal Dementia (tauopathies) MSA, PD, CBD, Lewy Body (synucleinopathies)MSA, PD, CBD, Lewy Body (synucleinopathies) HIVHIV MIDMID PrionopathiesPrionopathies –CJD –CJDv –Gerstmann-Straussler-Skenker –Fatal Familial Insomnia

167. Reversible Drugs –Alcohol, alcohol, alcohol Metabolic –B12 –T4 Infectious –Syphilis SOL –Subdural –Meningioma NPH Sleep Apnea Pseudodementia

168. Alzheimer's Disease 50 to 60% of cases of dementia.50 to 60% of cases of dementia. Greater than 65 years old -Greater than 65 years old - –prevalence 1-6%. 4th most common cause of death4th most common cause of death

169. Diagnostic Criteria "Probable (clinically ascertained) A.D. DementiaDementia Onset 40 to 90 yearsOnset 40 to 90 years Deficits present in greater than 2 cognitive spheresDeficits present in greater than 2 cognitive spheres Progressive deterioration.Progressive deterioration. No disturbance of consciousness.No disturbance of consciousness. No other illness to account for symptoms.No other illness to account for symptoms.

170. "Definite" (pathologicallv confirmed) AD Clinical criteria.Clinical criteria. Histopathological evidence.Histopathological evidence. –neurofibrillary tangles –neuritic plaques –granulovacular degeneration –Hirano bodies

171. Alzheimer’s Disease Pathophysiology Acetylcholine (memory)Acetylcholine (memory) –Nucleus Basalis of Meynert –diagonal band of Broca –medial septal nuclei

172. Multi-Infarct Dementia Greater than 50-100% of cerebral hemisphere destroyedGreater than 50-100% of cerebral hemisphere destroyed Multiple strokes involving both hemispheresMultiple strokes involving both hemispheres Bilateral pyramidal signsBilateral pyramidal signs Pseudobulbar signsPseudobulbar signs

173. Vitamin B12 Deficiency Insidious onsetInsidious onset May have normal smear and normal neurologic exam except for dementiaMay have normal smear and normal neurologic exam except for dementia Look for paresthesias plus signs of dorsal column and corticospinal tract involvement.Look for paresthesias plus signs of dorsal column and corticospinal tract involvement.

174. Normal Pressure Hydrocephalus TriadTriad –Ataxia –Incontinence –Dementia 6-12 months usually idiopathic CSF Pressure Measurements CT and RISA Rx - VP shunt

175. Depression " Pseudodementia" Commonly have history of previous psychiatric disorder.Commonly have history of previous psychiatric disorder. Brief duration.Brief duration. Complaint of cognitive deficit but make little effort to performComplaint of cognitive deficit but make little effort to perform even with simple tasks.even with simple tasks. Frequent "don't know" answers.Frequent "don't know" answers. Associated features of depression.Associated features of depression.

176. Creutzfeldt-Jacob Disease Onset to death usually months.Onset to death usually months. Dementia. myoclonus. UMN. basal ganglia.Dementia. myoclonus. UMN. basal ganglia. Characteristic EEG - periodic discharge 1/sec,Characteristic EEG - periodic discharge 1/sec, Caused by prionsCaused by prions

181. Environmental Factors Head traumaHead trauma Infectious agentsInfectious agents NeurotoxinsNeurotoxins AlcoholAlcohol

182. Down's Syndrome and AD Neuropathologic similaritiesNeuropathologic similarities Role of chromosome 21Role of chromosome 21

183. Investigation CT, MRICT, MRI EEGEEG B12. folateB12. folate CBC, differential. plateletsCBC, differential. platelets VDRLVDRL Thyroid function testsThyroid function tests BUN. creatinine.BUN. creatinine.

184. Investigation AST. ALTAST. ALT LytesLytes ESRESR CXRCXR Neuropsychological testingNeuropsychological testing Lumbar punctureLumbar puncture

185. Case 6 This 28 year old man presented with a three week history of a headache and weakness on the left side The day of admission he suffered a generalized seizure which was characterized by initial twitching to the left side of the face

186. Case 20 Neuro exam showsNeuro exam shows InattentiveInattentive Left facial weaknessLeft facial weakness Mild left upper and lower limb weakness and hyperreflexiaMild left upper and lower limb weakness and hyperreflexia Normal sensationNormal sensation Decreased RAM of left upper and lower limbsDecreased RAM of left upper and lower limbs

187. Where is the lesion?

188. CT Scan

189. Brain Tumors GBM

190. Meningioma

191. Brain Tumors Medulloblastoma

192. Tumor Often in frontal lobe.Often in frontal lobe. Grasp, suck. snout reflexesGrasp, suck. snout reflexes "slowness" in carrying out tasks"slowness" in carrying out tasks Impaired smellImpaired smell Tumors obstructing 3rd or 4th ventriclesTumors obstructing 3rd or 4th ventricles

193. Case 19 This 70 year old lady noted a 9 month history of tremors and clumsiness in both her hands and feetThis 70 year old lady noted a 9 month history of tremors and clumsiness in both her hands and feet Physical examPhysical exam –See video

195. Parkinson’s Disease 70 to 80 years old70 to 80 years old rarely less than 40 years oldrarely less than 40 years old 1/1.0001/1.000

196. Cardinal Features a. tremor.a. tremor. b. rigidityb. rigidity c. bradykinesiac. bradykinesia d. postural instabilityd. postural instability

197. Tremor resting tremorresting tremor "pill rolling""pill rolling" 5 to 6 Hz5 to 6 Hz unilateral earlyunilateral early increases with stressincreases with stress decreases with movementdecreases with movement

198. Rigidity "lead pipe""lead pipe" –bilateral – 1 side greater than the other

199. Bradykinesia Bradykinesia masked faciesmasked facies decreased blink frequencydecreased blink frequency decreased rapid alternating movementsdecreased rapid alternating movements decreased extraocular movementsdecreased extraocular movements hypophonia, palilalia. aprosodyhypophonia, palilalia. aprosody sialorrheasialorrhea micrographiamicrographia

200. Bradykinesia Bradykinesia decreased spontaneous movementdecreased spontaneous movement difficulty rising from chair or rolling over in beddifficulty rising from chair or rolling over in bed slow ADLslow ADL characteristic stooped gait with small stepscharacteristic stooped gait with small steps and decreased arm swingand decreased arm swing "freezing"freezing

201. Postural instability retropulsionretropulsion festinationfestination sit "en bloc"sit "en bloc" fallingfalling

202. Clinical Stages Stage I Mild unilateral tremor or rigidity with or without bradykinesiaMild unilateral tremor or rigidity with or without bradykinesia

203. Stage II Moderate bilateral tremor or rigidity and bradykinesia.Moderate bilateral tremor or rigidity and bradykinesia.

204. Stage III Significant tremor. rigidity and or bradykinesia plus impairedSignificant tremor. rigidity and or bradykinesia plus impaired postural reflexespostural reflexes gait disturbance, mild daily fluctuations +- dementiagait disturbance, mild daily fluctuations +- dementia

205. Stage IV Severely disabled but still mobile and able to functionSeverely disabled but still mobile and able to function independently - with or without daily periods of completeindependently - with or without daily periods of complete immobility; +- dementiaimmobility; +- dementia

206. Stage V Loss of ability to function independentlyLoss of ability to function independently

207. Autonomic dysfunction constipationconstipation dysphagiadysphagia neurogenic bladderneurogenic bladder droolingdrooling orthostatic hypotensionorthostatic hypotension diaphoresisdiaphoresis

208. Primary sensory symptoms vague parasthesiavague parasthesia

209. Etiology Abnormal processing of synuclein proteinAbnormal processing of synuclein protein ToxinsToxins Infectious agentsInfectious agents Immunological factorsImmunological factors Genetic factorsGenetic factors MPTPMPTP

210. Pathophysiology Progressive loss of presynaptic dopaminergic neurons in the substantia nigraProgressive loss of presynaptic dopaminergic neurons in the substantia nigra cortical pathological changes like AD in 50%.cortical pathological changes like AD in 50%. Changes in post synaptic striatal dopamine receptorsChanges in post synaptic striatal dopamine receptors

211. Treatment Mild Disability AnticholinergicAnticholinergic AmantadineAmantadine SelegilineSelegiline

212. Moderate Disability L-DopaL-Dopa RopineroleRopinerole PramipexolePramipexole

213. Severe Disability Increased doses of L-Dopa and Dopamine AgonistIncreased doses of L-Dopa and Dopamine Agonist COMT InhibitorCOMT Inhibitor

215. Long Term Complications of Treatment with L-Dopa DyskinesiaDyskinesia Daily fluctuations in level of functionDaily fluctuations in level of function –End of dose deterioration –freezing episodes –"on-off" phenomenon Dementia and drug induced confusionDementia and drug induced confusion Progressive drug failure.Progressive drug failure.

216. Case Study 34 year old woman with one year history of difficulties with voice and swallowing with weakness in right hand

218. Case Exam Cranial NervesCranial Nerves DysarthriaDysarthria Fasciculations of tongueFasciculations of tongue MotorMotor –Upper and lower limb weakness and wasting –Upper and motor limb hyperreflexia SensorySensory –Normal

219. Case Where is the lesion?Where is the lesion? What is the cause?What is the cause? What is the prognosis?What is the prognosis?

220. Amyotrophic Lateral Sclerosis (ALS) Natural History * Progressive asymmetrical muscular Wasting and weakness * Initially weakness begins in one or Two muscles * Adjacent muscles intact and Compensating for weakness Of involved muscles * Hyperexcitability of involved Muscles associated with Muscle cramps and fasciculation

221. Amyotrophic Lateral Sclerosis (ALS) Natural History * PROGRESSIVE COURSE LEADING TO DEATH * PROGRESSIVE COURSE LEADING TO DEATH MONTHS TO YEARS UNTIL COMPLETE MONTHS TO YEARS UNTIL COMPLETE PARALYSIS PARALYSIS * RANGE 1 TO 15 YEARS * RANGE 1 TO 15 YEARS * 50% DIE WITHIN 4 YEARS * 50% DIE WITHIN 4 YEARS * 20% LIVE FOR FIVE YEARS * 20% LIVE FOR FIVE YEARS * 10% LIVE FOR TEN YEARS * 10% LIVE FOR TEN YEARS * AGE LESS THAN 65 AVERAGE * AGE LESS THAN 65 AVERAGE DURATION OF LIFE 3 YEARS DURATION OF LIFE 3 YEARS * AGE GREATER THAN 65, AVERAGE * AGE GREATER THAN 65, AVERAGE DURATION OF LIFE 2 YEARS DURATION OF LIFE 2 YEARS

222. Amyotrophic Lateral Sclerosis Natural History * Apparent stabilization of the Disease may be compensation By other muscle groups * Age of onset more than 50 years Median age of onset 55 * Rarely develops before age 30 * Median age seems to be increasing

223. ALS Signs In pseudobulbar group, disorders of pursuit movements are common Minor losses of sensory function Little involvement of autonomic system Dementia is uncommon, unless age or Premiered dementia cause co-existence of ALS and dementia

224. ALS Signs * Atrophic weak limb with hyperreflexia * Mixture of upper and lower Motor neuron signs, Characteristic of bulbar form * Hyperactive jaw jerk with a sucking Reflex, common in bulbar form * Pseudobulbar emotional incontinence * Paralysis of extraocular movement or loss of bowel and bladder Continence

225. Amyotrophic Lateral Sclerosis Symptoms * No pattern to site of onset * Fatiguability early complaint * Hyperactive DTRs with spasticity

226. Amyotrophic Lateral Sclerosis Symptoms * Asymmetrical fatigue,cramping, Fasciculations, weakness And atrophy of muscles * Atrophied and normal muscles may Be found adjacent in the same limb * When disease begns in the muscles Of the tongue,lips and throat, Limb weakness is usually not present Initially but progresses later * Early recognition of bulbar symptoms

227. ALS Signs * Minor losses of sensory function * Little involvement of autonomic system * Dementia is uncommon,unless age or Premorbid dementia cause co-existence Of als and dementia

228. ALS Treatment * Supportive * Prevention of complications * Respiratory - Pneumonia - Tracheostomy - Ventilation * Nutrition - Feeding tube * Musculoskeletal - Splints - Contractures

229. ALS Treatment * Social - Acceptance of diagnosis - Early decisions re: trach and Ventilator - Support of dying patient

230. Case Study 30 year old male referred for evaluation Fell asleep while driving causing an accident Occurred in early afternoon Snores at night, witnessed apneas in sleep BMI 33.9 Normal - Neuro exam Thick neck, redundant soft palate

235. Obstructive Sleep Apnea Clinical Features Middle Aged Males Excessive Daytime Sleepiness (EDS) Snoring - Loud, Gutteral, Inspiratory Observed Respiratory Pauses in Sleep Irresistable Sleep Attacks Behavioral Automatisms With Amnesia Marked Nocturnal Movement Enuresis Morning Headache Late Cyanosis Polycythemia Edema Dyspnea Nocturnal Death Pickwickian Syndrome

237. The Sleep Apnea Syndromes Apnea defined as cessation of airflow at the nostrils and mouth lasting ten seconds or more Obstructive secondary to sleep induced airway obstruction Central apnea due to decrease activity of muscles of respiration Mixed apnea from a combination of both

238. Sleep Apnea Nasal-CPAP improves EDS Effect is objectively measurable with the multiple sleep latency test (MSLT) Epworth Score Increased Adult prevalence of sleep apnea/hypopnea syndromes is about 2-4%. Bed partner best witness Cofactors:BMI, use of alcohol,CNS depressants PSG confirmation

239. Risk Factors Obesity Micrognathia Enlarged Tonsils and Adenoids Enlarged Thyroid Acromegaly (enlarges tongue) Nasal Septal Defects Neuromuscular Disease Miscellaneous: Assoc’d With Narcolepsy-cataplexy (“-20%) Relation to Sudden Infant Death Syndrome

242. Management Causal –weight loss –removal of T and A –mandibular advancement surgery Relieve obstruction –continuous nasal positive pressure in sleep –uvolo-palato-pharyngoplasty –tracheostomy Drugs –medroxyprogesterone - (pure Pickwickians) Abstinence from alcohol, hypnotics, sedatives

245. Driving Recommendations for Patients With Obstructive Sleep Apnea Patients with obstructive sleep apnea (documented by a sleep study), who are compliant with CPAP or have had successful UPPP treatments should be safe to drive any type of motor vehicle.

246. Case Study 30 year old tow truck driver with history of EDS Episodes of uncontrolled sleepiness with paralysis Episodes of loss of posture with extreme emotion No family history

247. Case Study Neuro Exam – Normal HLA-DQB1*0602 – pending Patients receives Letter from MTO suspending licence – unable to work, no private insurance OSS and MSLT ordered

249. MSLT Pretreatment

250. MSLT Post treatment

251. Narcolepsy Cardinal symptoms –Sleep attacks and EDS –Cataplexy –Sleep paralysis –Vivid hypnagogic hallucinations Ancilliary symptoms –“Microsleeps” –Automatic behavior –Memory problems –Visual problems –Non-restorative night sleep –Nightmares

252. Narcolepsy Genetics HLA Linkage - DQB1*0602 – same HLA relationship has also been observed for essential hypersomnia (EHS).

254. Etiology Genetic and Sporadic Cases –Abnormal hypocretin receptorhypocretin –HLA DR 15 (DR2), DQB1*0602 Sporadic alone (60% of cases) –Precipitating Factors Irregular Prior Sleep/Wake Patterns Flu-like Illnesses Symptomatic Cases (Rare) –Demyelinating Disease –Tumoral –Post-traumatic (all in hypothalamus)

256. Treatment CNS Stimulants for EDS –methylphenidate –Amphethamines CNS Alerting Drugs –Modafinil REM Suppressants –tricyclics –clomipramine –desipramine –Imipramine –SSRIs –MAO inhibitors Experimental Drugs –gamma-hydroxybutyrate –zimelidine –naloxone

257. Driving recommendations for narcoleptic patients: Patients with a diagnosis of narcolepsy supported by a sleep study and with uncontrolled episodes of cataplexy during the past 12 months (with or without treatment) should not drive any type of motor vehicle. Patients with a diagnosis of narcolepsy supported by a sleep study and with uncontrolled daytime sleep attacks or sleep paralysis in the past 12 months (with or without treatment) should not drive any type of motor vehicle.

258. Occupational Risk Occurrence Sudden incapacitation – Cognitive Decline Psychomotor Slowing Secondary Complications

259. Occupational Risk Groups Low –Operators of Private Motor Vehicles –Office workers –Physicians –Retail Workers Intermediate –Taxis, ambulances, buses –Surgeons, EMT –Mechanics, electricians High –Pilots, divers, Drivers Class A, B, C, D –Chemical, nuclear industry –Operators of weapons of mass destruction

260. Huntington’s Disease Prevalence 5-10/100,000Prevalence 5-10/100,000 Motor. cognitive and behavioural manifestationsMotor. cognitive and behavioural manifestations Mean age of onset 36 years.Mean age of onset 36 years. Duration 19 years.Duration 19 years. Autosomal dominant with complete penetranceAutosomal dominant with complete penetrance 10-15% -juvenile onset10-15% -juvenile onset

261. Huntington’s Disease Westphal variantWestphal variant 90% from affected father90% from affected father 10-15% - greater than 55 years old10-15% - greater than 55 years old slower declineslower decline

262. Neuropathology Caudate and putamenCaudate and putamen Atrophy. neuronal depletion. gliosisAtrophy. neuronal depletion. gliosis Decreased GABA and acetylcholineDecreased GABA and acetylcholine

263. Gene Defect Short arm of chromosome 4Short arm of chromosome 4

264. Coma A. AirwayA. Airway B. BreathingB. Breathing C. CirculationC. Circulation Neurological ExaminationNeurological Examination 1. Respiration1. Respiration 2. Pupils, oculomotor function2. Pupils, oculomotor function 3. Skeletal motor3. Skeletal motor

265. Respiration Bilateral encephalopathy - Cheyne StokesBilateral encephalopathy - Cheyne Stokes Upper pens - hyperventilationUpper pens - hyperventilation Pontine tegmentum - apneustic breathingPontine tegmentum - apneustic breathing Lower pons - cluster breathingLower pons - cluster breathing Meduila - ataxic breathingMeduila - ataxic breathing

266. Pupils Bilateral hemisphere diencephalonBilateral hemisphere diencephalon –Small reactive III nerve.III nerve. –Uncal mass with herniation Tectal - large fixedTectal - large fixed Midbrain - mid position. regular fixedMidbrain - mid position. regular fixed Pons -Small pinpoint.Pons -Small pinpoint.

267. Extra-Ocular Movements conjugateconjugate deviateddeviated skewskew bobbing; nystagmusbobbing; nystagmus

268. Reflex eye movements Doll's eyesDoll's eyes Calorics (COWS)Calorics (COWS) Dysconjugate -MLFDysconjugate -MLF Lost - brainstem nucleiLost - brainstem nuclei

269. Motor Responses HemisphereHemisphere –appropriate ipsilateral –flexion contralateral Hypothalamus and midbrainHypothalamus and midbrain –decorticate Lower midbrain -Lower midbrain - –decerebrate Medulla -Medulla - –Flexion of upper limbs. –rudimentary flexion of lower limbs.

270. Differential Diagnosis of Coma Supratentorial mass lesions.Supratentorial mass lesions. Midbrain or pontine destructive lesions.Midbrain or pontine destructive lesions. Intratentorial mass lesions.Intratentorial mass lesions. Diffuse multifocal disorders.Diffuse multifocal disorders. PseudocomaPseudocoma

271. Investigations Glucose, BUN. creatinine,Glucose, BUN. creatinine, Gas. ASTGas. AST Lytes Lytes Toxic screenToxic screen CTCT EEGEEG LPLP

272. Seizures alcohol withdrawal seizuresalcohol withdrawal seizures 12 to 48 hrs following cessation of alcohol intake12 to 48 hrs following cessation of alcohol intake generalized tonic clonic seizuresgeneralized tonic clonic seizures 2-3 seizures2-3 seizures risk of delerium tremensrisk of delerium tremens

273. Neurology of Alcoholism RUM fitsRUM fits Seizure induced by alcoholSeizure induced by alcohol

274. Seizure Induced by Alcohol usually focalusually focal reflect intrinsic CNS diseasereflect intrinsic CNS disease often post traumatic due to multiple fallsoften post traumatic due to multiple falls rule out subdural hematoma and meningitisrule out subdural hematoma and meningitis

275. Wernickes Encephalopathy Due to Thiamine Deficiency Ocular changes Ocular changes –nystagmus – 6th nerve palsy –paralysis of conjugate gaze AtaxiaAtaxia ConfusionConfusion

276. Korsokoff's Psychosis extension of confusion of Wernickesextension of confusion of Wernickes permanent severe memory impairment with confabulationpermanent severe memory impairment with confabulation

277. Treatment Thiamine 100 mg IVThiamine 100 mg IV Repeat daily until patient is back on normal diet.Repeat daily until patient is back on normal diet. Intravenous glucose - always give with thiamineIntravenous glucose - always give with thiamine - glucose depletes thiamine stores and- glucose depletes thiamine stores and can give rise to Wernickes encephalopathycan give rise to Wernickes encephalopathy

278. Polyneuropathy Polyneuropathy Nutritional and toxicNutritional and toxic Distal weakness and paresthesiaDistal weakness and paresthesia TreatmentTreatment –diet –abstinence from alcohol –multivitamins

279. Cerebellar Degeneration males more than femalesmales more than females trunchal ataxia and lower limb dysmetriatrunchal ataxia and lower limb dysmetria TreatmentTreatment –diet and vitamins. –abstinence from alcohol

280. Delerium Tremens 72 to 96 hours72 to 96 hours Severe tremulousnessSevere tremulousness Hallucinations - visual and auditoryHallucinations - visual and auditory autonomic hyperactivity - hypertension. fever. dilatedautonomic hyperactivity - hypertension. fever. dilated pupils and diaphoresispupils and diaphoresis Can be fatalCan be fatal

281. Treatment ThiamineThiamine FolateFolate Lorazepam, diazepamLorazepam, diazepam

282. Duchenne Muscular Dystrophy (DMD) Commonest lethal x-linked dystrophy 1/4,000 live male births Delayed motor development 1/2 unable to walk by 18 months Waddle Lordosis Problem running and climbing stairs Toe walking Frequent fall

283. Duchenne Muscular Dystrophy Gower’s manoeuvre Proximal weakness Calf hypertrophy Hyporeflexia Wheelchair by 7 to 12 years Contracture scoliosis Obesity or cachexia Death by teens or early 20’s from respiratory or cardiac complications Lower IQ with 1/3 mentally retarded Cardiomyopathy