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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
•Physical Exam•Fever 38.0 C• Inattentive•Poor short term memory•Left upper quadrantopsia•Hyperflexia Left upper and lower limb
Case
Case 1
• Where is the lesion?– why?
• What is the cause?• What are the immediate treatment
priorities?
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
Herpes SimplexTreatment
• Acyclovir IV• Management of ICP (max at 8 – 10 Days)
– Head up– Hyperventilation– Mannitol– Hypertonic Saline
• Seizure treatment
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 ?
CT Scan
without contrast
Subarachoid Hemorrhage
CT ScanSubarachnoid
Blood
Subarachnoid Hemorrhage
• Worse headache of life• Sudden onset, often with activity• Signs of meningeal irritation
– Kernig, Brudzinski
• Focal signs• Signs of coma• Positive CT scan• Positive LP
Subarachnoid Hemorrhage
• Blood in subarachnoid space• Require urgent referral for angiogram• Use acetaminophen not ASA for
headache
Subarachnoid HemorrhageInvestigations
• CT Scan - 90 - 95% sensitive• LP - nearly 100% sensitive
– rbc in CSF– xanthochromic in CSF after 12 – 18 hours
• Angiogram• Treatment
– surgical clipping– coiling
Subarachnoid HemorrhageTreatment
• Clipping• Coiling
Giant Aneurysm
GDC Coil
Headache in the Emergency Room
1. Distinguish ominous from benign headache
2. Treat effectively the benign headaches
Assessment ApproachAirway
Breathing
Circulation
Drugs
Evaluation
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
The Spectrum of Ominous Headaches
• Subarachnoid hemorrhage• meningitis• increased intracranial pressure
Danger Signals
• the worse headache ever• onset with exertion• decreased level of consciousness• meningeal irritation• abnormal physical signs (including fever)• worsening
All Clear Signals
• previous identical headaches• patient bright and alert• neck supple
– Kernig, Brudzinski’s signs
• normal examination• improving without analgesics
CT Scan
• detects most conditions causing increased ICP
• misses 10 - 15 % of subarachnoid hemorrhage
• misses nearly all cases of meningitis
Modified HIS Diagnostic Criteria for Migraine
• multiple previous attacks• duration of attacks a few hours to a few days• headaches have at least two of:
• hemicranial• severe• pulsating• worse with activity
• headaches accompanied by at least one of:• nausea and or vomiting• aversion to light or noise
• no evidence of ominous disease in history or examination
Classification of Primary Headaches
• Migraine–with aura, without aura–Ophthalmoplegic, retinal
• Tension Headache• Cluster Headache• Miscellaneous without structural
lesion
Treating Migraine in the ER
1. Restore intravascular volume
2. Identify contraindications
3. Choose appropriate medication
Analgesic - Antinauseant
• Toradol• Metoclopramide • IV slow push
DHE - Antinauseant
• Metoclopramide 10 mg IV, followed in 10 minutes by
• DHE 0.5 - 1.0 mg IV by slow push
Chlorpromazine• Ensure patient is normovolemic
– 250 - 500 cc crystalloid
• Prepare 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 when – improvement clearly occurring, or– 25 mg given– Watch for hypotension and sedation
Nasal Sprays
• DHE
• Sumatriptan
Sumatriptan
1 mg SC if:• no ergot or DHE in past 24 hours• no contraindication
Patient Instructions:Guidelines
• Do not drive a car or operate machinery• Do not drink alcohol or take
tranquillizers, antihistamines, or other drugs that affect the CNS
• Store in child-resistant containers• Neurological followup if frequent or
incapacitating
Cluster Headache
• 1/10 as common as migraine• Not genetically determined• M:F 6:1• Later Onset• Rhythmicity• Severe unilateral orbital, supraorbital
and/or temporal pain
Cluster Headache
• Ipspilateral– conjunctival injection– nasal congestion– forehead and facial swelling– miosis– ptosis– eyelid edema
• Every 2 - 8 times per day
Cluster Headache
• Trigger– alcohol
• Pathophysiology– Role of proximal internal carotid artery– Role of histamine
• Treatment• Acute attack
– ergotamine
Cluster Headache
• Prophylaxis–Sansert–Steroids–Lithium–Calcium channel blockers–Combinations
Inflammatory Headaches
• Most people who think they have sinus headaches usually have migraine or muscle contraction headache
• Diagnosis requires acute sinusitis• Nasal congestion, post nasal drip, fever,
pain over the involved sinuses• Tender on percussion• Sinus xray confirms• Treatment
– Antibiotics or drainage
Temporal Arteritis
• Progressively obliterative granulomatous arteritis
• Temporal or occipital• Can involve cerebral and ophthalmic
arteries• 50% will go blind and have a stroke• Greater than > years
Temporal Arteritis
• Usually temporal headache• Malaise, anorexia, night sweats, myalgia,
+/- fever, jaw claudication• ESR > 50• Diagnosis
– Superficial temporal artery biopsy• Treatment
– Prednisone 60 -100 mgm daily
Meningeal Irritiation
• Meningitis and subarachnoid hemorrhage
• Occurs due to inflammation and intracranial pain sensitive
• structures.• Subarachnoid hemorrhage - sudden
onset meningitis• Meningitis - more gradual.
Meningeal Irritiation
• Occipital and nuchal pain.• Interscapular and low back pain.• Aggravated by movements• Photophobia, vomiting• Fever• Diagnosis• History and physical• CT LP
Headache and Brain tumor
• Traction on intracranial pain sensitive structures.
• Progressively worsening headache.• Morning headaches.• Worsen in head down position.• Worsen with cough and straining• May be localized (constant).• Focal neurologic signs.
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
Hierarchy of Prevention
Risk Factors – Headache Diary
Alcohol/Foods/Sleep deprivation/Meals/Stress management/Repetitive Stress injury
Amitriptyline/Beta blockers/pizotyline/Singulair
Lithium/DBS
Valproate/Chlorpromazine
Hierarchy of Treatment
Acetaminophen/NSAIDS
Muscle Relaxants/Topiramate/Valproate
Narcotics/Steroids/Oxygen
Triptans/Chlorpromazine
Case
• 22 year old man develops double vision especially when looking up or to either side• He has noted some increased fatiguability of his muscles• EOM shown in video1• Exam otherwise normal
Case
• He is then given 10 mgm of IV Tensilon (Edrophonium)
• His extra ocular movements are then reexamined
•Video 2
Case
• Where is the lesion?
• What other tests might be used?
• What is the commonest treatment?
• What are the long term risks of the disease?
Myasthenia GravisDefinition
* Autoimmune disease with destruction Of neuromuscular junctions
* Symptoms
- Progressive fatigibility over time
Myasthenia GravisSymptoms
* Progressive fatigibility over time
* Double vision
* Drooping of eyelid(s)
* Difficulty swallowing
* Change in voice
* Difficulty breathing
Myasthenia GravisSigns
* Weakness of eyes movements
* Weakness of facial muscles
* Weakness of swallowing, cough
Or gag
* Weakness of limbs
* No sensory loss
Myasthenia Gravis
Myasthenia GravisInvestigations
• Tensilon Test
• EMG
• Anti-acetylcholine receptor antibodies
Myasthenia GravisTreatment
• Mestinon (pyridostigmine)
• Steroids
• IV IgG
• Plasma exchange
• Thymectomy
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 ?
CT Scans
Left ACA and MCA Territory Infarction
CT Scans
Left Carotid Stenosis
Angiogram Pathology
Stroke
• 50,000 new cases per year• #3 cause of death
Risk Factors
• age - doubles every decade after 55 years• sex - males 25% greater than females• blood pressure - 5 times• Cholesterol• Sleep apnea – 3 –4 times• smoking - 4 times• alcohol abuse - 3 times• diabetes - 3 times• homocystein
Definition
• Transient ischemic attack– Focal cerebral ischemic event resolving
within 24 hours
• Completed Stroke– No resolution of symptoms
Causes of Stroke
• 85% infarct -– 60% cerebral atherosclerosis– 20% lacunar– 15 cardiogenic emboli
• 15% hemorrhage – – intracerebral– subarachnoid hemorrhage
Cerebral Circulation 2 Vertebral Arteries Basilar• 2 Carotid Arteries• Circle of Willis
CT Angiogram
Vascular Distribution
Lacune
• Fibrinoid degeneration of penetrating arteries
• Most commonly due to hypertension• Involves deep white matter pens and
basal ganglia
Cardiogenic Emholi
• NVAF -45%.• IHD - acute MI -15%• - ventricular aneurysm -10%• RHD -10%• Prosthetic valve -10%• Other -10%.
Stroke SyndromesCarotid Circulation
Internal Cerebral Artery
• Hemiplegia• Hemisensory loss• Aphasia (L)• Neglect (R)
Anterior Cerebral artery• Contralateral lower limb paresis and
hyperreflexia• upper limb relatively spared
PCA Infarct
Vertebrobasilar Circulation
• Multiple stroke syndromes depending on vessels in site
• Dysarthria• Ataxia• Bilateral weakness• Bilateral sensory complaints• Bilateral visual complaints
Pontine InfarctLocked-In Syndrome
Lacunar Infarct
• Multiple stroke syndromes but in general produces pure motor or pure sensory stroke
Isolated Symptoms Rarely due to Stroke or TIA
• Vertigo. dizziness• Diplopia• Loss of consciousness• Confusion
Differential Diagnosis
• Transient Ischemic attack• Focal seizure• Hypoglycemia• Carpal tunnel syndrome• Migraine• Hysteria
Ddx Vertebrobasilar TIA
• Syncope• Labyrinthitis• Myasthenia gravis• Meniere's disease
Investigation• CT, CTA, CT perfusion • MRI/MR angiogram • CBC. differential and platelets• INR, PTT• Cholesterol, triglyceride• Doppler• Echocardiogram
Vertebrobasilar Circulation
• As above but no Doppler• Treatment• Prevention:• Controlled risk factors
Carotid Circulation
• If no severe carotid stenosis or cardiac embolus• antiplatelet agents. specifically aspirin, Plavix
or Ticlid.• Cardiac embolus is treated with Coumadin• Carotid stenosis severe than 70% is treated
with carotid endarterectomy or angioplasty and stenting
• Risk factor management
Acute Thrombolysis
Pre
Post
Treatment of Acute Stroke
• NINDS protocol– < 4.5 hours since onset– < 1/3 of MCA territory– No recent bleeding or surgery– IV r-tpa and/or intraarterial tpa angioplasty,
stent
Brain AttackDistribution of Hemorrhages
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
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 #
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
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
Skull fracture
epiduralWhat would be the symptoms and signs? What would be the treatment?
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.
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.
Acu
te o
n c
hro
nic
SD
HWhat would be the symptoms and signs? What would be the treatment?
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.
Subdural hematoma
• Drowsiness. headache• Evolves over days to weeks• History of head trauma not always
present
Subdural Hematoma
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?
Multiple SclerosisMRI
Demyelinating DiseaseClassification
• Multiple sclerosis.• Diffuse cerebral sclerosis.• Post viral or post vaccinial disseminated
encephalomyelitis.• Necrotizing hemorrhagic encephalomyelitis.• Metabolic toxic• postanoxic encephalopathy• CPM
Dysmyelinating Disorders
• ALD• MLD• Krabbe's• Canavan's• Chediak Higashi• Neuraxonal dystrophy• Pelazeus Merzbacher
Multiple Sclerosis
• Dissemination of lesions in time and space• MacDonald Criteria• EDSS or Kurtze Scale• Prevalence 0.1%• Female:male = 3:2• 20 to 40 years old (peak age 28 years)
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
Practical use of MRI in the diagnosis and management of patients with
MS
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
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
Lesions in TimeClinical 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
Paraclinical TestsMRI
Most sensitive and specific
Visual evoked potentials Can be used as objective clinical evidence Delayed with preserved wave form
CSF required for:Equivocal MRIPPMS diagnosisUnusual clinical picture
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
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
Definite MS (DMS) Requirements:
2 Clinical attacks2 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 attack2 objective signs
1 objective sign
1–3 MRIs required Gd lesion > 3 months after clinical attack
orNew T2 or Gd lesion > 3 months after 1st MRI
2–3 MRIs required Positive 1st MRI AND Gd lesion > 3 months
after clinical attack
Treatment• Acute Attacks• Steroids.
– Solumedrol– Prednisone
• Prophylaxis– Beta interferon– Copolymer– Mitoxandrone– Natalizumab– Fingolimod (Gilenya)– Teriflunomide (Aubagio)– Dimethyl fumarate (Tecfidera)
Symptomatic
• Spasticity– Baclofen– Dantrium– Benzodiazepines
• Pain– Gabapentin– Pregabalin– Carbamazepine
Treatment
• Paroxysmal Disorders– Tegretol– Dilantin
• Bladder Dysfunction– Anticholinergic drugs eg. (Ditropan)– Antibiotic treatment
• Depression– Tricyclic antidepressants– SSRI– SNRI
• Fatigue– Amantidine
Seizures
• Seizures originate from the cortex• Case
Simple Partial Seizures.
1. motor
2. somatosensory or special sensory
3. autonomic
4. psychic
Complex Partial Seizures
• Partial seizure with altered awareness• SP -CP• CP• + automatisms.
Partial Seizures - to secondary generalization
• SP-GTC• CP - GTC• SP -CP-GTC
Generalized Seizures (convulsive and non convulsive)• Absence
– Typical– Atypical
• Myoclonic• Clonic• Tonic• Tonic-clonic• Atonic.
History• How did the seizure start
– staring– eye deviation– jerking or one limb
• Patient's description of aura• Post ictal state• Age of onset• Family history• Past history• Alcohol or drug abuse
Physical Exam
• Todds paralysis• Eye deviation• Cranial bruit• Hyperventilation• Skin examination• Incontinence
Investigation
• Glucose. BUN, Calcium. Sodium• CT, MRI• EEG
TreatmentPartial Seizures
• Carbamazepine• Phenytoin• Primidone• Phenobarbital• Valproic Acid• Lamotrigine• Levetiracetam• Lacosamide
Tonic Clonic
• Valproic acid (Divalprpoex)• Carbamazepine (Tegretol)• Phenytoin (Dilantin)• Phenobarbital• Primidone (Mysoline)• Levetiracetam (Keppra)• Lacosamide (Vimpat)
Absence
• Valproate• Ethosuximide
Myoclonic
• Valproate• Clonazepam• Nitrazepam
Add On Meds
• Gabapentin• Lamictal• Vigabatrin• Topiramate
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.
• On examination, he has general mental slowing with some tremor in the left upper limb.
• Where is the lesion ?
CT Scan
Dementia• A loss of intellectual ability of sufficient severity
to interfere with social or occupational functioning.
• Memory impairment.• At least one of:
– impaired abstract thinking– Impaired judgement– Other disturbances of higher cortical functioning -– aphasia. apraxia, agnosia. constructional difficulty– Personality change
Irreversible• Alzheimer’s Disease (amyloidopathies) • Frontotemporal Dementia (tauopathies)• MSA, PD, CBD, Lewy Body (synucleinopathies)• HIV• MID
– Large vessel– Small vessel
• Prionopathies– CJD– CJDv– Gerstmann-Straussler-Schenker– Fatal Familial Insomnia
Reversible• Drugs
– Alcohol, alcohol, alcohol• Metabolic
– B12– T4
• Infectious– Syphilis
• SOL– Subdural– Meningioma
• NPH• Sleep Apnea• Pseudodementia
Alzheimer's Disease
• 50 to 60% of cases of dementia.• Greater than 65 years old -
– prevalence 1-6%.
• 4th most common cause of death
Diagnostic Criteria"Probable (clinically ascertained)
A.D.• Dementia• Onset 40 to 90 years• Deficits present in greater than 2
cognitive spheres• Progressive deterioration.• No disturbance of consciousness.• No other illness to account for symptoms.
"Definite" (pathologicallv confirmed) AD
• Clinical criteria.• Histopathological evidence.
– neurofibrillary tangles– neuritic plaques– granulovacular degeneration– Hirano bodies
Alzheimer’s DiseasePathophysiology
• Acetylcholine (memory)– Nucleus Basalis of Meynert– diagonal band of Broca– medial septal nuclei
Multi-Infarct Dementia
• Greater than 50-100% of cerebral hemisphere destroyed
• Multiple strokes involving both hemispheres
• Bilateral pyramidal signs• Pseudobulbar signs
Vitamin B12 Deficiency
• Insidious onset• May have normal smear and normal
neurologic exam except for dementia• Look for paresthesias plus signs of dorsal
column and corticospinal tract involvement.
Normal Pressure Hydrocephalus
• Triad– Ataxia– Incontinence– Dementia
• 6-12 months• usually idiopathic• CSF Pressure Measurements• CT and RISA• Rx - VP shunt
Depression" Pseudodementia"
• Commonly have history of previous psychiatric disorder.
• Brief duration.• Complaint of cognitive deficit but make
little effort to perform• even with simple tasks.• Frequent "don't know" answers.• Associated features of depression.
Creutzfeldt-Jacob Disease
• Onset to death usually months.• Dementia, myoclonus, UMN, basal
ganglia.• Characteristic EEG - periodic discharge
1/sec,• Caused by prions
Environmental Factors
• Head trauma• Infectious agents• Neurotoxins• Alcohol
Down's Syndrome and AD
• Neuropathologic similarities• Role of chromosome 21
Investigation
• CT, MRI• EEG• B12. folate• CBC, differential. platelets• VDRL• Thyroid function tests• BUN. creatinine.
Investigation
• AST. ALT• Lytes• ESR• CXR• Neuropsychological testing• Lumbar puncture
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
Case 20
• Neuro exam shows• Inattentive• Left facial weakness• Mild left upper and lower limb weakness
and hyperreflexia• Normal sensation• Decreased RAM of left upper and lower
limbs
Where is the lesion?
CT Scan
Brain TumorsGBM
Meningioma
Brain TumorsMedulloblastoma
Tumor
• Often in frontal lobe.• Grasp, suck. snout reflexes• "slowness" in carrying out tasks• Impaired smell• Tumors obstructing 3rd or 4th ventricles
Case 19
• This 70 year old lady noted a 9 month history of tremors and clumsiness in both her hands and feet
• Physical exam– See video
Parkinson’s Disease
• 70 to 80 years old• rarely less than 40 years old• 1/1.000• Video 1• Video 2• Video 3• Video 4
Cardinal Features
• a. tremor.• b. rigidity• c. bradykinesia• d. postural instability
Tremor
• resting tremor• "pill rolling"• 5 to 6 Hz• unilateral early• increases with stress• decreases with movement
Rigidity
• "lead pipe"– bilateral– 1 side greater than the other
Bradykinesia
• masked facies• decreased blink frequency• decreased rapid alternating movements• decreased extraocular movements• hypophonia, palilalia. aprosody• sialorrhea• micrographia
Bradykinesia
• decreased spontaneous movement• difficulty rising from chair or rolling over
in bed• slow ADL• characteristic stooped gait with small
steps• and decreased arm swing• "freezing
Postural instability
• retropulsion• festination• sit "en bloc"• falling
Clinical StagesStage I
• Mild unilateral tremor or rigidity with or without bradykinesia
Stage II
• Moderate bilateral tremor or rigidity and bradykinesia.
Stage III
• Significant tremor. rigidity and or bradykinesia plus impaired
• postural reflexes• gait disturbance, mild daily fluctuations
+- dementia
Stage IV
• Severely disabled but still mobile and able to function
• independently - with or without daily periods of complete
• immobility; +- dementia
Stage V
• Loss of ability to function independently
Autonomic dysfunction
• constipation• dysphagia• neurogenic bladder• drooling• orthostatic hypotension• diaphoresis
Primary sensory symptoms
• vague parasthesia
Etiology
• Abnormal processing of synuclein protein
• Toxins• Infectious agents• Immunological factors• Genetic factors• MPTP
Pathophysiology
• Progressive loss of presynaptic dopaminergic neurons in the substantia nigra
• cortical pathological changes like AD in 50%.
• Changes in post synaptic striatal dopamine receptors
TreatmentMild Disability
• Anticholinergic• Amantadine• Selegiline
Moderate Disability
• L-Dopa/Carbidopa• Pramipexole (Mirapex)• Ropinoral (Requip)
Severe Disability
• Increased doses of L-Dopa and Dopamine Agonist
• COMT Inhibitor
Long Term Complications of Treatment with L-Dopa
• Dyskinesia• Daily fluctuations in level of function
– End of dose deterioration– freezing episodes– "on-off" phenomenon
• Dementia and drug induced confusion• Progressive drug failure.
Case Study
• 34 year old woman with one year history of difficulties with voice and swallowing with weakness in right hand
Case Exam
• Cranial Nerves• Dysarthria• Fasciculations of tongue
• Motor– Upper and lower limb weakness and wasting– Upper and motor limb hyperreflexia
• Sensory– Normal– Video
Case
• Where is the lesion?
• What is the cause?
• What is the prognosis?
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
Amyotrophic Lateral Sclerosis (ALS)
Natural History * PROGRESSIVE COURSE LEADING TO DEATH MONTHS TO YEARS UNTIL COMPLETE PARALYSIS
* RANGE 1 TO 15 YEARS
* 50% DIE WITHIN 4 YEARS
* 20% LIVE FOR FIVE YEARS
* 10% LIVE FOR TEN YEARS
* AGE LESS THAN 65 AVERAGE DURATION OF LIFE 3 YEARS
* AGE GREATER THAN 65, AVERAGE DURATION OF LIFE 2 YEARS
Amyotrophic Lateral SclerosisNatural 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
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
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
Amyotrophic Lateral SclerosisSymptoms
* No pattern to site of onset
* Fatiguability early complaint
* Hyperactive DTRs with spasticity
Amyotrophic Lateral SclerosisSymptoms
* 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
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
ALSTreatment
* Supportive
* Prevention of complications
* Respiratory - Pneumonia - Tracheostomy - Ventilation
* Nutrition - Feeding tube
* Musculoskeletal - Splints - Contractures
ALSTreatment
* Social
- Acceptance of diagnosis - Early decisions re: trach and Ventilator - Support of dying patient
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
Obstructive Sleep ApneaClinical Features
Middle Aged MalesExcessive 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
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
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
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
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
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.
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
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
MSLT Pretreatment
MSLT Post treatment
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
Narcolepsy
• Genetics• HLA Linkage - DQB1*0602
– same HLA relationship has also been observed for essential hypersomnia (EHS).
Etiology
• Genetic and Sporadic Cases– Abnormal hypocretin receptor– 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)
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
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.
Occupational RiskOccurrence
• Sudden incapacitation – • Cognitive Decline• Psychomotor Slowing• Secondary Complications
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
Huntington’s Disease
• Prevalence 5-10/100,000• Motor. cognitive and behavioural
manifestations• Mean age of onset 36 years.• Duration 19 years.• Autosomal dominant with complete
penetrance• 10-15% -juvenile onset
Huntington’s Disease
• Westphal variant• 90% from affected father• 10-15% - greater than 55 years old• slower decline
Neuropathology
• Caudate and putamen• Atrophy. neuronal depletion. gliosis• Decreased GABA and acetylcholine
Gene Defect
• Short arm of chromosome 4
Coma
• A. Airway• B. Breathing• C. Circulation• Neurological Examination• 1. Respiration• 2. Pupils, oculomotor function• 3. Skeletal motor
Respiration
• Bilateral encephalopathy - Cheyne Stokes• Upper pens - hyperventilation• Pontine tegmentum - apneustic breathing• Lower pons - cluster breathing• Meduila - ataxic breathing
Pupils
• Bilateral hemisphere diencephalon– Small reactive
• III nerve. – Uncal mass with herniation
• Tectal - large fixed• Midbrain - mid position. regular fixed• Pons -Small pinpoint.
Extra-Ocular Movements
• conjugate• deviated• skew• bobbing; nystagmus
Reflex eye movements
• Doll's eyes• Calorics (COWS) • Dysconjugate -MLF• Lost - brainstem nuclei
Motor Responses• Hemisphere
– appropriate ipsilateral– flexion contralateral
• Hypothalamus and midbrain– decorticate
• Lower midbrain - – decerebrate
• Medulla - – Flexion of upper limbs.– rudimentary flexion of lower limbs.
Differential Diagnosis of Coma
• Supratentorial mass lesions.• Midbrain or pontine destructive lesions.• Intratentorial mass lesions.• Diffuse multifocal disorders.• Pseudocoma
Investigations
• Glucose, BUN. creatinine, • Gas. AST• Lytes• Toxic screen• CT• EEG• LP
Seizures
• alcohol withdrawal seizures• 12 to 48 hrs following cessation of alcohol
intake• generalized tonic clonic seizures• 2-3 seizures• risk of delerium tremens
Neurology of Alcoholism
• RUM fits• Seizure induced by alcohol
Seizure Induced by Alcohol
• usually focal• reflect intrinsic CNS disease• often post traumatic due to multiple falls• rule out subdural hematoma and
meningitis
Wernickes EncephalopathyDue to Thiamine Deficiency
• Ocular changes– nystagmus– 6th nerve palsy– paralysis of conjugate gaze
• Ataxia• Confusion
Korsokoff's Psychosis
• extension of confusion of Wernickes• permanent severe memory impairment
with confabulation
Treatment
• Thiamine 100 mg IV• Repeat daily until patient is back on normal
diet.• Intravenous glucose - always give with
thiamine• - glucose depletes thiamine stores and• can give rise to Wernickes encephalopathy
Polyneuropathy
• Nutritional and toxic• Distal weakness and paresthesia• Treatment
– diet– abstinence from alcohol– multivitamins
Cerebellar Degeneration
• males more than females• trunchal ataxia and lower limb dysmetria• Treatment
– diet and vitamins.– abstinence from alcohol
Delerium Tremens
• 72 to 96 hours• Severe tremulousness• Hallucinations - visual and auditory• autonomic hyperactivity - hypertension.
fever. dilated• pupils and diaphoresis• Can be fatal
Treatment
• Thiamine• Folate• Lorazepam, diazepam
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
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
Back Pain
• 33 year old left handed HVAC worker• Develops left hip pain in Oct 2012, treated
with NSAIDs and rest• Recurrence summer 2013 when diving into
lake• January 2014, sudden left leg pain and
numbness radiation anterolateral aspect of left leg
History
• Treated conservatively with physio and chiropractor
• Physical Exam– Weakness L plantar flexor, loss of ankle jerk
Localization
• Muscle• Neuromuscular Junction• Peripheral Nerve• Spinal Cord