LMCC Preparation
Back to Basics
Neurology
Dr. C.R. Skinner
14 April 2010
Major Topics
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Neurology Made Simple Review
Headache
Trauma
Infections
Cerebrovascular Disease
Demyelination
Seizures
Degenerative Diseases
Sleep Disorders
Neurology Made Simple
Questions
• Is the Problem Neurological?
• If So , Where Is It in the Nervous
System ?
• What Is the Most Likely Cause ?
• Is This Problem Serious Enough to
Require Urgent Referral ?
• How to Stabilize the Patient During
Transport
NEUROLOGY MADE SIMPLE
IS THE PROBLEM NEUROLOGICAL?
 Are there hard organic features ?
 Is the behaviour bizarre ?
 Is there a history of seizures, drug
addiction or of psychiatric illness ?
 Do the signs and symptoms make sense ?
NEUROLOGY MADE SIMPLE
INTRODUCTION
NEUROLOGICAL PROBLEM
WHERE IS THE LESION
WHAT IS THE CAUSE
INVESTIGATION
TREATMENT
PROGNOSIS
Localization Matrix
Muscle
Mental Status
Cranial Nerves
Motor
Reflexes
Sensory
Coordination
Gait
Neuromuscular
Junction
Deep White
Peripheral Spinal Brain Matter/Basal
nerve
Cord Stem Ganglia/Thalamus Cortex Cerebellum
NEUROLOGY MADE SIMPLE
INTRODUCTION
NEUROLOGICAL PROBLEM
WHERE IS THE LESION
WHAT IS THE CAUSE
INVESTIGATION
TREATMENT
PROGNOSIS
Etiology Matrix
Drugs
Acute
Subacute
Chronic
Inflammatory Metabolic Vascular Electrical Trauma Neoplastic Degenerative Genetic
NEUROLOGY MADE SIMPLE
INVESTIGATIONS
 History
 Physical
 According to presumed localization
and etiology
 Metabolic
systemic
CSF
 Structural
 Neurophysiological
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?
ESSENTIAL HISTORICAL FEATURES
HISTORY OF PRESENT ILLNESS
Headache
Loss Of Consciousness
Weakness
Difficulty With Vision , Hearing ,
Taste
Numbness
Weakness
Dizziness
Lightheadedness , Vertigo, Off
balance
Fatigue
Loss Of Balance
Loss Of Coordination
Difficulty Swallowing, Chewing
Urinary And Stool Incontinence
Sexual Dysfunction
Sleep Difficulties
Etiology Matrix
Drugs
Acute
Subacute
Chronic
Inflammatory Metabolic Vascular Electrical Trauma Neoplastic Degenerative Genetic
NEUROLOGY MADE SIMPLE
LOCALIZATION ANALYSIS
 Muscle
 Neuromuscular junction
 Peripheral nerve
 Spinal cord
 Brain stem
 Thalamus or basal ganglia
 Cortex
 Cerebellum
Localization Matrix
Muscle
Mental Status
Cranial Nerves
Motor
Reflexes
Sensory
Coordination
Gait
Neuromuscular
Junction
Deep White
Peripheral Spinal Brain Matter/Basal
nerve
Cord Stem Ganglia/Thalamus Cortex Cerebellum
Muscle
Neuromuscular Junction
Peripheral Nerves
Lateral Medullary Syndrome
• Ipsilateral
– Pain numbness, impaired
sensation over half of face
– Ataxia of limbs with
falling towards side of
lesion
– Vertigo, nausea, vomiting
– Horner’s syndrome
• Contralateral
– Impaired pain and
temperature sensation
over half of the body and
Lateral Pontine Syndrome
• Ipsilateral
– Horizontal, vertical
nystagmus vertigo, nausea,
vomiting, oscillopsia
– Facial paralysis
– Paralysis of conjugate gaze
to side of lesion
– Deafness, tinnitus
– Ataxia
– Impaired sensation over face
• Contralateral
– Impaired pain and thermal
sense over half of body
Ventral Midbrain Syndrome
Weber’s Syndrome
• Ipsilateral
• Diplopia, dilated
pupil
• Ataxia
• Contralateral
• Hemiplegia,
mainly upper
limb and face
CORRELATIVE FACTORS
CAPSULE, THALAMUS AND BASAL GANGLIA
•
Hemi-sensory or motor signs
•
Sensory signs of primary modalities
•
Sensory involvement of the trunk
•
Lack of cortical signs
•
Uniform motor signs in arm ,
leg and face
•
Hypertension risk factor
CORRELATIVE FACTORS
CORTICAL
•
Aphasia and right sided weakness
fluent, non - fluent, paraphasia
•
Weakness - arm and face more than leg
•
Visual field defects
•
Cortical sensory disturbance
inattention
left-right
acalculia
agnosia
apraxia
Cerebellum
Basal Ganglia
•Modulating structures
•Rule out other systems first
•Ipsilateral Rule for pure
cerebellar lesions
FINAL CHECKLIST
THINGS NOT TO MISS
 MUSCLE
POLYMYOSITIS, POLYMYALGIA RHEUMATICA
 NEUROMUSCULAR
MYASTHENIA
 PERIPHERAL NERVE
GUILLAIN - BARRE SYNDROME
 SPINAL CORD
ACUTE SPINAL CORD COMPRESSION
FINAL CHECKLIST
THINGS NOT TO MISS
BRAIN STEM
STROKE, MULTIPLE SCLEROSIS
MENINGITIS
LISERIA, TB
GUILLAIN - BARRE - FISHER VARIANT
TUMORS
FINAL CHECKLIST
THINGS NOT TO MISS
 CORTEX
 STROKE
 HERPES ENCEPHALTIS
 SEIZURES
 TUMORS
 SUBARACHNOID HEMORRHAGE
Circulation
du LCR
Circulation
of CSF
Case
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 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 Simplex
Treatment
• 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 Scan
Subarachnoid
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 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
Subarachnoid Hemorrhage
Treatment
• Clipping
• Coiling
Giant Aneurysm
GDC Coil
Headache in the Emergency
Room
1. Distinguish ominous from benign
headache
2. Treat effectively the benign headaches
Assessment Approach
Airway
Breathing
Assess, secure
Not a problem unless obtunded
Assess, assist
Not a problem unless obtunded
O2 not necessary
Circulation
Drugs
IV line with crystalloid to restore volume
No drugs until diagnosed, unless required to
stablize circulation
In particular, no analgesics until diagnosed
Evaluation
Rapid neurological assessment
Investigations as necessary
The Spectrum of Ominous
Headaches
• Subarachnoid hemorrhage
• meningitis
• increased intracranial pressure
Danger Signals
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•
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•
•
•
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
Narcotic - Antinauseant
• Meperidine 75 - 100 mg plus
• Prochlorperazine 5-10 mg
• 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
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•
•
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•
•
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
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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 Treatment
Narcotics/Steroids/Oxygen
Triptans/Chlorpromazine
Muscle Relaxants/Topiramate/Valproate
Acetaminophen/NSAIDS
Hierarchy of Prevention
Lithium/DBS
Valproate/Chlorpromazine
Amitriptyline/Beta blockers/pizotyline/Singulair
Risk Factors – Headache Diary
Alcohol/Foods/Sleep deprivation/Meals/Stress management/Repetitive Stress
injury
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 video
• Exam otherwise normal
Case
• He is then given 10 mgm of IV Tensilon
(Edrophonium)
• His extra ocular movements are then
reexamined
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 Gravis
Definition
* Autoimmune disease with destruction
Of neuromuscular junctions
* Symptoms
- Progressive fatigibility over time
Myasthenia Gravis
Symptoms
* Progressive fatigibility over time
* Double vision
* Drooping of eyelid(s)
* Difficulty swallowing
* Change in voice
* Difficulty breathing
Myasthenia Gravis
Signs
* Weakness of eyes movements
* Weakness of facial muscles
* Weakness of swallowing, cough
Or gag
* Weakness of limbs
* No sensory loss
Myasthenia Gravis
Myasthenia Gravis
Investigations
• Tensilon Test
• EMG
• Anti-acetylcholine receptor antibodies
Myasthenia Gravis
Treatment
• 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
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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
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•
•
•
•
•
NVAF -45%.
IHD - acute MI -15%
- ventricular aneurysm -10%
RHD -10%
Prosthetic valve -10%
Other -10%.
Stroke Syndromes
Carotid Circulation
Internal Cerebral Artery
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•
•
•
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 Infarct
Locked-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
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•
•
•
Vertigo. dizziness
Diplopia
Loss of consciousness
Confusion
Differential Diagnosis
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Transient Ischemic attack
Focal seizure
Hypoglycemia
Carpal tunnel syndrome
Migraine
Hysteria
Ddx Vertebrobasilar TIA
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Syncope
Labyrinthitis
Myasthenia gravis
Meniere's disease
Investigation
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CT, CTA, CT perfusion
MRI/MR angiogram
CBC. differential and platelets
INR, PTT
Cholesterol, triglyceride
Doppler
Echocardiogram
Vertebrobasilar Circulation
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•
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
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–
–
–
< 3 hours since onset
< 1/3 of MCA territory
No recent bleeding or surgery
IV r-tpa and/or intraarterial tpa angioplasty,
stent
Brain Attack
Distribution 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:
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–
–
–
–
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
What would be the symptoms and signs? What would be the treatment?
epidural
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 extraaxial 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.
Acute on chronic SDH
What 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 craniocerebral 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 Sclerosis
MRI
Demyelinating Disease
Classification
• Multiple sclerosis.
• Diffuse cerebral sclerosis.
• Post viral or post vaccinial disseminated
encephalomyelitis.
• Necrotizing hemorrhagic encephalomyelitis.
• Metabolic toxic
• postanoxic encephalopathy
• CPM
Dvsmvelinating 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 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
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
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 attacks
0–1 MRI required
2 objective signs
None ― but recommended
(Caution if MRI and CSF are normal)
1 objective sign
3 of 4 Barkhof criteria
or 2 MRI lesions and abnormal CSF
1 Clinical attack
2 objective signs
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
1 objective sign
Positive 1st MRI AND Gd lesion > 3 months
after clinical attack
Treatment
• Acute Attacks
• Steroids.
– Solumedrol
– Prednisone not for optic neuritis
• Prophylaxis
–
–
–
–
Beta interferon
Copolymer
Mitoxandrone
Natalizumab
• Symptomatic
• Spasticity
– Baclofen
– Dantrium
– Benzodiazepines
Treatment
• Paroxysmal Disorders
– Tegretol
– Dilantin
• Bladder Dysfunction
– Anticholinergic drugs eg. (Ditropan)
– Antibiotic treatment
• Depression
– Tricyclic antidepressants
• 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
Treatment
Partial Seizures
•
•
•
•
•
Carbamazepine
Phenytoin
Primidone
Phenobarbital
Valproic Acid
Tonic Clonic
•
•
•
•
•
Valproic acid
Carbamazepine
Phenytoin
Phenobarbital
Primidone
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, 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 Disease
Pathophysiology
• 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 Tumors
GBM
Meningioma
Brain Tumors
Medulloblastoma
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
Cardinal Features
•
•
•
•
a.
b.
c.
d.
tremor.
rigidity
bradykinesia
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 Stages
Stage 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
Treatment
Mild Disability
• Anticholinergic
• Amantadine
• Selegiline
Moderate Disability
• L-Dopa
• Ropinerole
• Pramipexole
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
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 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
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 Sclerosis
Symptoms
* No pattern to site of onset
* Fatiguability early complaint
* Hyperactive DTRs with spasticity
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
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
ALS
Treatment
* Supportive
* Prevention of complications
* Respiratory
- Pneumonia
- Tracheostomy
- Ventilation
* Nutrition
- Feeding tube
* Musculoskeletal
- Splints
- Contractures
ALS
Treatment
* 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 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
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 Risk
Occurrence
•
•
•
•
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 Encephalopathy
Due 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