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					LMCC Preparation
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      Neurology
   Dr. C.R. Skinner
    14 April 2010
               Major Topics
•   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
                                                                  Deep White
                          Neuromuscular   Peripheral Spinal Brain Matter/Basal
                 Muscle   Junction        nerve      Cord Stem Ganglia/Thalamus Cortex Cerebellum



Mental Status



Cranial Nerves



Motor



Reflexes



Sensory



Coordination



Gait
NEUROLOGY MADE SIMPLE
       INTRODUCTION

  NEUROLOGICAL PROBLEM

  WHERE IS THE LESION

  WHAT IS THE CAUSE

  INVESTIGATION

  TREATMENT

  PROGNOSIS
                                 Etiology Matrix


           Drugs   Inflammatory Metabolic Vascular Electrical Trauma Neoplastic Degenerative Genetic




Acute




Subacute




Chronic
    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   Inflammatory Metabolic Vascular Electrical Trauma Neoplastic Degenerative Genetic




Acute




Subacute




Chronic
NEUROLOGY MADE SIMPLE
  LOCALIZATION ANALYSIS
 Muscle

 Neuromuscular junction

 Peripheral nerve

 Spinal cord

 Brain stem

 Thalamus or basal ganglia

 Cortex

 Cerebellum
                              Localization Matrix
                                                                  Deep White
                          Neuromuscular   Peripheral Spinal Brain Matter/Basal
                 Muscle   Junction        nerve      Cord Stem Ganglia/Thalamus Cortex Cerebellum



Mental Status



Cranial Nerves



Motor



Reflexes



Sensory



Coordination



Gait
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 CSF
    Circulation of
              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       Assess, secure
              Not a problem unless obtunded
Breathing     Assess, assist
              Not a problem unless obtunded
              O2 not necessary
Circulation   IV line with crystalloid to restore volume
  Drugs       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
•   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
•   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 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
•   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 Syndromes
           Carotid 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 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
•   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
  –   < 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:
  –   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 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.
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 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 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      1–3 MRIs required
   2 objective signs      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.   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 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                    Pickwickian
   Edema                           Syndrome
   Dyspnea
   Nocturnal Death
     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

				
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