Launch Neurology Toronto …launchneurologytoronto.com/images/Launch Neurology Toronto.pdfApraxia of...
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1. Behavioral neurology
a) How do you test for calculation?
b) Name two prefrontal lobe tests?
c) How do you test for apraxia? How is apraxia of left frontal lobe lesions different from that of
left parietal lobe lesions?
2. Epilepsy
a) Define Drug resistance and Epilepsy remission and Seizure freedom?
b) What advise will you give a patient to prepare for a sleep deprived EEG?
c) What is hemolytic disease of the newborn? How do you prevent it?
d) 20 female with a seizure.
1) What questions will you ask to identify the type of seizure disorder?
2) Patient has symptoms of both myoclonic and absence seizures. What investigation will you
do?
3) Patient insists for the best medication. What will you give?
4) When you described the issues with VPA with pregnancy, she is asking for an alternative.
What will you give? How will you prescribe it?
5) You started her on LTG. If the patient wants to become pregnant and if patient agrees with a
prescription of LTG, what all things you would like to discuss?
6) What is the mechanism behind the need for increased dose of LTG?
7) “If we keep the concentration appropriate, will LTG cause any seizures doctor?”
8) You prescribed LTG. Patient cannot sleep well at night. How do you manage?
9) After delivery she stated to take is on OCP. How does that affect LTG?
3. Headache
a) How will you treat severe headache in pregnancy?
b) What are the FDA drug categories?
c) What are the features of aura in Migraine with aura?
d) How do you treat acute SUNCT in ER? What prophylaxis can you prescribe?
4. Internal Medicine
a) Patient with history of chronic alcoholism. Now has double vision for the last 1 week. What is
the most common diagnosis? How do you manage?
1) What typical questionnaire you will administer?
2) Where will you refer this patient?
3) How do you manage if this patient become delirious in the hospital after 2 days?
4) What is the typical doze of diazepam for alcohol withdrawal delirium?
5) How do you manage post-operative delirium?
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b) List viruses causing predominantly encephalitis and meningitis
c) 60 Male with acute onset seizures once at home. H/o HTN, smoking. History & exam. Cough,
loss of weight, bone pain, mild unilateral weakness & lesion on CT brain at the gray-white
junction with edema. How do you manage?
5. Movement disorder
a) Palatal tremor
1) Palatal tremor (Watch a video) Where is the lesion?
2) What are the types of palatal tremors?
3) How do you treat palatal tremor?
4) What is common accompanying change in the eyes?
b) Examine a person with parkinsonian features
c) DBS how do you select a candidate for DBS and what locations will you select?
6. MS/Inflammatory
a) What are the relapse rates of MS during pregnancy and post-partum periods compared to the
normal?
b) What are the differential diagnosis for very large white matter lesions?
c) What are the diagnostic criteria for NMO spectrum disorders?
7. Neuromuscular
a) What are the three typical features of ICU myopathy and explain why?
b) How do you differentiate between pseudobulbar palsy from bulbar palsy?
c) What special history and examination will you carry out in a case of myopathy?
d) How to you test myotomes of upper and lower limbs? What muscles will you test for L5
myotome?
8. Neuro-Ophthalmology
a) Name nuclei associated with horizontal and vertical eye movements?
b) 48 F has diplopia. O/E abnormal lid elevation with any ocular movements for more than a few
months. What is the name of the condition? What are the different types? What investigations
will you order?
c) A patient has nystagmus. How do you differentiate between central and peripheral
nystagmus?
9. Pediatric Neurology
a) Name three neurological disorders that has high chances to appear sporadically and mention
the percentage chances? What other mode of transmission do each one of them have?
b) A patient diagnosed with Neurofibromatosis type 2 does not have any vestibular schwanoma.
Can this be correct? Yes or No. Substantiate your answer.
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c) A child with mild mental impairment show neck extension, nodding, some features of
spasmodic torticollis that last only for a few minutes. What specific history do you want to
ask? Is it occurring while eating?
d) Differentiate between Typical v/s Atypical absence seizures?
e) Write a list of diseases affected by AD, AR, XD and XR pattern of inheritance?
10. Stroke
a) What conditions give number needed to treat (NNT) values 8, 6, 4 and 2?
b) Name four conditions that can cause Moyamoya disease?
c) What investigations will you do for stroke in a young patient?
d) Patient on warfarin. Now INR is high. How do you manage?
11. Telemedicine
a) Name three neurological disorders where NSAIDs are contraindicated?
b) GBS-like ascending paralysis is seen in which electrolyte abnormality?
c) Relapse rates in MS
1. MS patient who don’t want to take any medications
2. MS patient who is taking disease modifying drugs
3. MS during 3rd trimester
4. MS during postpartum period
ANSWERS
1. Behavioral neurology
a) Ask patient to calculate 6+8-7. Note that simple addition or subtraction are not enough.
b) Prefrontal lobe tests
1) Wisconsin Card Sorting Test: Color, form, number - challenges the subject to shift cognitive
sets without warning. Poor cognitive flexibility & perseveration will show up.
2) Stroop test (Read the print of the name of a color printed with a different color)
c) Praxis testing
Check for handedness
“I will make sure that vision, hearing, attention and orientation are normal before testing for
apraxia” Hearing or vision problem – Yes/No questions; 3 step command
“I WILL RULE OUT ABNORMALITIES IN STRENGTH, CO-ORDINATION, BALANCE &
SENSATIONS”
Test 1 proximal & 1 distal group muscles in each limb
Gross coordination – FNF; Fine coordination: pick up a pen
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Balance – stand up on one leg for a couple of seconds
Sensation: Close your eyes – which hand am I touching now?
I am looking to see if the person is using correct body parts, making correct movements in
correct spatial orientation & with correct speed & repetition of movement where relevant
Ideomotor – Left parietal
Upper limb- meaningless gesture, pantomime a salute (show it), Show how you brush your teeth
with your right hand, Use it wrongly & ask to command.
How do you use this correctly – act to me? How you really use it?
Lower limb – kick a ball
Whole body – stand like a boxer
Bucco-facial apraxia – stick out your tongue, blow a kiss, blow out a match, drink using a straw
Apraxia of speech – pa, ta, ka, pataka, pataka, Repeat pencil 3 times,
Hopeful-hopefully, Spagatti, Episcopalian
Constructional tasks – copy a cross, triangle with a triangle, draw a flowerpot, design blocks
Gait apraxia – ask to walk
Only left hand affected – Anterior corpus callosum – Geshwind
Ideomotor (Only motor) Left parietal lesion - has anosognosia & left frontal lesion - can
recognize the defect
Ideational – Entire left hemisphere
Fold a letter, put it in an envelope, seal, stamp, address & mail 2) Light a candle
Ideational (Planning): Entire left hemisphere. Can’t do sequence (Can’t make a sandwich)
Imitation problem – Right hemisphere
Limb Kinetic Apraxia – Cannot unbutton the shirt. Cannot pick a coin from the table
2. Epilepsy
Theory
a) Definitions
1) Drug resistance = failure of adequate trials of 2 appropriately chosen, tolerated &
administered anti-seizure drugs (whether as monotherapy/ in combination) to achieve
seizure freedom
2) Epilepsy remission: 10 years seizure free with last 5 years off AEDs + passed the age of
epilepsy syndromes
3) Seizure freedom: "Rule of 3": Seizure free after an intervention period = 3x the largest pre-
intervention inter-seizure interval or 12 months, whichever is longer
b) Reduce sleep by one hour both at the beginning and at the time of getting up
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c) Hemorrhagic disease of the newborn is due to the side effect of enzyme inducing anti-seizure
medications that the mother is taking during pregnancy. It can be prevented by prescribing
Vitamin K 10mg OD PO during last trimester to the mother and by giving the newborn a one
time injection of 1 mg vitamin K
d) 19 year old female with seizures
1) Ask for symptoms of myoclonic (exaggerated with reduced sleep and alcohol), atypical
absences (see pediatric question 9d), generalized tonic clonic seizure and other types of
seizures
2) EEG routine with photic stimulation & hyperventilation and sleep deprived EEG
3) Valproic acid is the best medication for both myoclonic and absences
4) LTG. Start 25 BID x 1week, slowly increase to 100 BID (over 6 weeks) (Max 150/200
BID). Get a blood level, if seizure not controlled after 100 BID to further increase doze
5) Check blood levels of LTG before becoming pregnant and it will become necessary to
increase the dose of LTG during pregnancy to maintain same blood level. Follow up this
during the postpartum period
6) Mechanism: Increased estrogen especially during third trimester activates uridine glucuronyl
transferase (UGT) & lowers LTG
7) LTG may worsen myoclonus in some patients: tell this to your patient
8) LTG has some stimulating effect. So give the last dose of the day before 4 pm
9) Same as the answer to 6). This time it is due to oestrogen in the pill. Patient may again need
more LTG. Maintain the pre-pregnancy level
3. Headache
a) Severe headache in pregnancy Rx: Metoclopramide, Tylenol, Hydration if needed. [Tryptan in
pregnancy: Not recommended normally. But can be given. No teratogenic effects are reported.
Commonest side effect is atonia of uterus and so it can cause increased uterine bleeding. If
needed during lactation: Bottle feed baby for several hours after taking tryptan]
b) FDA drug categories
A: Human studies failed to show any defect in the 1st trimester
B: Animal studies – no defect – Clopidogrel
C: Animal studies – defect – ASA
D: Human fetal risk: VPA (but has to compare risk & benefit when treated)
X: Humans or animals bad
c) Features of aura in Migraine with aura
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1/< of fully reversible symptoms: Visual, Retinal, Sensory, Motor, Speech & /language, Brain
stem
At least two of following 4 characteristics
1. At least 1 aura symptom spreads gradually over 5 min &/or 1/ more symptoms occur in
succession.
2. Each individual aura symptom lasts 5–60 min.
3. At least 1 aura symptom is unilateral.
4. Aura is accompanied/ followed within 60 min, by headache (any type). Not better accounted
for by another ICHD-3 diagnosis & TIA has been excluded
d) Acute SUNCT Rx and prophylaxis
IV Lidocaine 1.3-3.3mg/kg/h. It has 100% cure rate
Prophylaxis by either Topiramate or LTG
4. Internal Medicine
a)
1) Wernicke’s encephalopathy. IV Thiamine 100 to 500 mg IV stat. Then daily x 3 days
2) CAGE questions
Have you ever thought of cutting down?
Have people annoyed you by criticizing your drinking?
Have you ever felt guilty?
Do you need an eye opener in the morning hours?
3) Alcoholic Anonymous
4) Diazepam IV
College of Family Physicians of Canada:
Schedule Day 1 Day2 Day 3 Day 4
Rigid 10 mg four times daily 10 mg three
times daily
10 mg twice
daily
10 mg at bedtime
Flexible 10 mg every 4–6
hours as needed based
on symptoms to a
maximum of 60
mg/day
10 mg every 6–8
hours as needed
10 mg every
12 hours as
needed
10 mg at bedtime
as needed
UpToDate: 5 to 10 mg IV every 5 to 10 minutes, until the appropriate level of sedation is
achieved. Studies have shown large efficacy of the loading dose method corresponding to
substantial reduction of the psychosis duration.
5) Rx: Haloperidol
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b)
Encephalitis (enveloped viruses) Meningitis
HSV-1 Rx: Acyclovir HSV-2 (Mollaret’s) Aseptic meningitis
Rx: Acycolovir
Arbo – Flavi (Culex) – JBE (Extrapyramidal,
Flacid paralysis, Rhombencephalitis), West
Nile (Back pain, tremor, flaccid paralysis), St.
Louis, Western Equine
Entero – Coxackie, Echo, Polio
Mumps
VZV (+ ASA = Rey’s syndrome)
LCM (lymphocytic choreomeningitis)
c) ABCs; Oxygen-IV-Monitor; CXR, CT head, EEG
Labs: Na 120
At this stage, I want to know vitals & blood sugar. Serum osmolality, Urine Na (24 hr urine),
volume status (I/O chart)
Serum electrolytes, serum osmolarity, Blood glucose, protein, EKG, urine lytes
SIADH: Na <135; Blood osmolarity <275 mosm/kg; urine osmolarity ~ 100mos; Urine Na
>40 meq/L. (Note: Urine 24 hr total volume osmolality 500-800; Random urine osmolality vary
between 50-1200; Normal urine Na = 20 meq/L)
SIADH has euvolemia & hyponatremia: Rx: Treat SIADH by water restriction
No AED even though Na is low. When patient’s sodium is very low and if patent start seizures
then sodium level has to be corrected using hypertonic saline
Regarding rate of hypertonic solution (3% NaCl; Normal saline is 0.9% NaCl)
Never let sodium level increase more than 10 meq in 24 hours
Calculate how many meq of NaCl is required to increase Na concentration up to 130 meq/L &
give that volume of 3% NaCl in 24 hours. (3% NaCl = 513 meq/L)
Body wt (60) x (130-120) x 50% (% lean body weight in men) = 60x10x0.5 = 300 meq
3% NaCl = 513 meq/L. Therefore, need 585 mL – give in 24 hours (so ~22 ml/hour)
Where is sodium balance sensed in the brain? Organum vasculosum of lamina terminalis
(OVLT) (no BBB)
How do you approach to hyponatremia? Na < 135 mmol/L
Is it acute or chronic? (Only acute is worrisome)
If patient is seizing, are seizures due to hyponatremia or something else?
What important additional lab values you want to have?
Plasma osmolality (Normal: 275 – 295 mOsm/kg)
Urine Na concentration (look for values <20 mmol/L or >20 mmol/L)
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If development of hyponatremia occurred within 48 hours, then the brain cells have not had time
to develop idiogenic osmoles. Commonly occurs in hospitalized patients who receive hypotonic
fluids postoperatively
Can Rx the hyponatremia more aggressively- Chance of osmotic demyelination syndrome
namely Central pontine myelinolysis (CPM) is there
If not seizing, use Normal saline to correct Na + salt tablets
Raise serum Na+ no more than 0.5 to 1.0 mmol/L/hr & by less than 10-12 mmol/L in 24 hrs
Check lytes q 2 h - until Na+ reach ~ 125 mmol/L
If seizing use (hypertonic saline) 3% saline solution
Na+ should not be ↑more than 10-12 mmol/L over the first 24 hours
A loop diuretic may be added to enhance water excretion if urine osmol is greater than 300
mOsm/kg
Rx with hypertonic saline solution is advocated only for patients with severe hyponatremia who
have profound neurologic symptoms
What is your approach in a case of high serum osmolality?
If hyperglycemia or hyperglycinemia: Rx these to Rx hypoNa & Rx Sz
If glucose & glycine are normal & has hyperlipidemia/ hyperproteinemia: pseudo-
hyponatremia If glucose, glycine, lipids, protein are normal: true hyponatremia
For example, hyperosmolality, due to hyperglycemia, can result in continuous partial seizures.
Seizures will respond to lowering the glucose
Volume status Hypovolemic Euvolemic Hper-volemic
Urine Na
>20mmol/L
Renal loss-diuretics,
Addison, salt losing
nephritis
Osmotic diuresis –
glucose, urea
SIADH
HypoT4
Low cortisol
Acute water overload –
Stress, post Sx,
polydypsia
Renal failure
Urine Na
<20mmol/L
Extra-renal loss
Vomiting, diarrhea, skin
loss
SIADH Rxed with fluid
restriction, fluid
depletion
Renal Na retention –
cirrhosis, CHF,
nephrotic syndrome
Rx:
Hypovolemic: Symptomatic give 3% saline; asymptomatic - normal saline
Euvolemic: Symptomatic give 3% saline judiciously + furosemide; asymptomatic - normal saline
+ oral salt tablets
Hypovolemic: Normal saline + furosemide; asymptomatic-furosemide
If seizing patient in ER: Tumor, stroke, SDH, head injury, HSV, CVST, hemorrhage into
tumor, hypo Na or hypo Mg
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If patient also has elevated CK. How to manage high CK? Minor degree of CK normalizes after
seizure cessation. If levels are high, forced diuresis can be used
Also look for signs of stroke, headaches, history of falls, constitutional symptoms, cognitive
changes?
Rx: General: DVT prophylaxis. Discuss palliative option with the patient or family regarding
palliation. Poor prognosis is associated cerebral mets from lung cancer
Palliative medicine consult for comfort measures
Seizures will be treated as any other complications
5. Movement disorder
a) Palatal tremor
1) Inferior Olivary N. The lesion may be very small and therefore, it may not be seen in a MRI
scan immediately. However, after 6 months, there will be pseudo-hypertrophy at the Inferior
Olivary nucleus visible in MRI (see a MRI)
2)
Primary (Essential) Secondary (Symptomatic) (After a stroke)
HARD TO DIAGNOSE – NEED A MRI!
Take an Elevator!
27% m=f 73% m>f
Age of onset 30 (M=F); eyelids affected 45 (M>F); eyelids not affected
Ear click present Absent
Tensor veli palatine affected Levator veli palatini affected
Possible Present in sleep - mostly
Olivary pseudo-hypertrophy (after 6 months)
Asso. with CNS signs, ataxia
3) Rx: Clonazepam 1mg HS
4) Has pendular nystagmus of 1Hz (watch a video)
b) Parkinsonian features
General examination: Postural BP changes: Check pulse and BP simultaneous at 3 postures
measured between 3 minutes between each of them.
Write name (micrographia), Draw a circling spiral
Parkinson’s Unified Parkinson disease rating scale (UPDRS) has 3 components:
1) Cognitive: mentation, behavior & mood, thought disorder, depression, motivation/initiative
2) ADLs + IADLs: Speech, salivation, swallowing, handwriting, cutting food/handling utensils,
dressing, hygiene, turning in bed/adjusting bed clothes, freezing when walking, falling
unrelated to freezing, tremor, sensory complaints related to PD
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3) Motor Exam: speech, facial expression, tremor at rest (4 limbs), if not seen, ask to name
months backwards, action or postural tremor (4 limbs), look fro re-emergent tremor, rigidity
(4 limbs), finger taps (upper limbs), hand movements (open & close) (upper limbs: rapid
alternating movements (pronate & supinate), (lower limbs: leg agility (tap on ground 3 inch),
rise from chair with hands folded in front, posture when standing up, gait, body bradykinesia/
hypokinesia, postural stability (Retropulsion test: 2 steps backwards are normal). Always
stand against a wall to prevent falls.
What specific care will you provide?
Medical Alert Bracelet
Power of attorney (health directives)
Referral to OT/PT for fall prevention, hypersalivation,
Driving (Clinical Dementia Rating scores 0.5, 1, 1.5, 2), inform MOT if needed
c) DBS
1) PD with good response to levodopa,
Severe motor fluctuations, dyskinesia, painful dystonia, side effect of meds, N/V, psychiatric
Age <75, interested in DBS,
No cognitive decline, can lie down for a MRI without any phobia
DBS – will improve dopamine sensitive motor symptoms
2) Locations for DBS
Ventral intermediate nucleus of the thalamus (VIN) for tremor
Globus Pallidus (GPi) for dystonia
STN (for tremor, rigidity, akinesia, postural istability) (TRAP for PD)
6. MS/Inflammatory
a)
Normal: 1%
3rd trimester: 0.2
Postpartum: 1.2 (high relapse) – Note lactation can help slightly. So advise breast feeding
Disease modifying drugs: 0.5%
b) Differential for very large white matter lesions
Celiac disease Anti-gliadin antibody; Tissue trans glutaminase
(TTG)
Paraneoplastic X-ray chest, Antibodies
CADASIL (AD) Notch 3 gene defect
Fatty acid oxidation & organic acid metab Acyl carnitine profile (free & total)
Abetalipoproteinemia Abetalipoprotein
Fabry’s Alpha 1 galactosidase
SCA 1,3 CAG repeats
Metachromatic leukodystrophy
MRI: All brain; Butterfly + Tiger skin
Blood arylsulfatase A + Urine sulphatide
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Adrenoleukodystrophy (posterior brain)
XR, Defect in ABCD1 gene, Diagnosis by
assay in skin fibroblast/ mutation analysis
Nerve Bx: demyelination + axonopathy
Rx: Steorids+Lorenzo oil+Gene therapy
Plasma ↑VLCFA (defect in its beta oxidation)
Na low, K high, high ACTH (adrenal problem)
Has peripheral neuropathy + UMN signs
(In old age ALD; if in young – D/d: Friedereick’s)
Sural N: Demyelination+Axon damage, onion
bulb
Leigh’s syndrome (SNE – subacute
necrotising encephalitis), MELAS
Mitochondrial disease tests
c) NMO spectrum disorders
I. The diagnosis of NMOSD with AQP4-IgG antibodies requires all the 3
1. At least one core clinical characteristic
2. A positive test for AQP4-IgG using the best available detection method (cell-based assay
strongly recommended)
3. Exclusion of alternative diagnoses
6 core criterial includes
1. Optic neuritis
2. Acute myelitis
3. Area postrema syndrome: episode of hiccups or nausea and vomiting
4. Acute brainstem syndrome
5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical
diencephalic MRI lesions
6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions
II. Criteria for NMOSD with negative or unknown AQP4-IgG antibody status
1. At least two core clinical characteristics occurring as a result of one or more clinical
attacks and meeting all of the following requirements:
• At least 1 core clinical characteristic must be optic neuritis, acute myelitis with LETM,
or area postrema syndrome
• Dissemination in space (2/ more different core clinical characteristics)
2. Negative tests for AQP4-IgG (NMO-IgG) using best available detection method
3. Exclusion of alternative diagnoses
7. Neuromuscular
a)
EMG will be normal. EMG examines Type 1 fibers (moves when patient moves a muscle).
Steroid myopathy affect type 2 fibers. So EMG will be normal
CK will be normal. This is because in steroid myopathy cell membranes are not leaky to CK.
So CK cannot escape from muscle to blood stream
Muscle biopsy shows loss of myosin. Steroid will cause myosin to lose from muscle
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b)
Pseudobulbar (UMN) Bulbar (LMN)
Affect mainly 5, 7; (but also 10, 11,12) Affect only 10, 11, 12
Affect cortico-bulbar tracts above the
nucleus. Usually supply bilaterally
At or below nucleus
↑GAG Reduced GAG
Spastic tongue without atrophy Fasciculation & wasting of tongue
↑jaw jerk
Affect mastication & facial expression
Jaw jerk normal (because 5 & 7 are not
affected)
Speech - spastic dysarthria If unilateral - raspy voice: Get MRI/MRA/CTA
If affect both sides – nasal speech, when try to
swallow water, it will come through the nose.
Emotions - labile Normal
Cause: Bilateral CVA affecting bilateral IC
(genu), MS, high brainstem tumors, head
injury
MND - ALS, Syringobulbia, GBS, Polio,
subacute meningitis (carcinoma, lymphoma),
neurosyphilis, brainstem CVA
c) Muscle weakness special history and exam
History
Cola colored urine? myoglobinuria
Worsen with exercise? MG or periodic paralysis
Is there any myotonia? Difficulty in relaxing after a hand shake?
Proximal muscle weakness: Difficulty rising from chairs, getting out of the bathtub, climbing
stairs, and/or shaving or combing the hair
Weakness of distal muscles: Weak grasp, handwriting problems, walking difficulties, trip over
Exam: Ask patient to change into a gown to examine the pattern of muscle wasting
Upper limb: Shoulder Extension, Flexion, External Rotation, Finger flexion
Lower limb: Abduction, adduction
Neck flexion
Always do: Myotonia percussion
Examine for calf hypertrophy, scapular winging
Test for hand grip and finger flexion
Functional testing: Running, stand up, squatting
Palpate for muscle tenderness
Test for myotonia? Percuss (fairly hard) on the muscle belly (not tendon) with knee hammer
d) Myotome testing
1) Testing myotomes of upper and lower limbs
C5 Elbow flexion L2 Hip flexion
C6 Wrist extension L3 Knee extension
C7 Elbow extension L4 Ankle dorsiflexion (stand on heel)
C8 Distal finger IP joint flexion L5 Big toe dorsiflexion
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T1 Little finger abduction S1 Ankle plantar flexion (stand on toes)
2) L5 myotome testing = Test all L5 muscles: TA, TP, PL, GM, BF (long & short head), ST, SM
8. Neuro-Ophthalmology
a) Nuclei
Horizontal eye movement: Nucleus prepositus hypoglossi & medial vestibular nucleus
Vertical movement: Interstitial nucleus of Cajal, Ri MLF, Posterior commissure
b) Lid elevation with ocular movements
If the eyelid shows abnormal elevation with any ocular movements, it means aberrant
regeneration. This condition is called synkinesis.
Types a) Muscle to lid: IR to lid - lid close when look down; SR to lid - lid close when look up;
MR to LPS; SR to LPS b) Muscle to pupil - Pupil constrict when that muscle is used
It is necessary to rule out tumor immediately. MRI Brain, MRI sella (to see parasellar masses –
giant aneurysms, pituitary adenoma, meningioma, craniopharyngioma) and MRI orbit
c) Differentiating between Central & peripheral nystagmus
It is carried out doing HiNTs test
Hi Head impulse (Halmagyi head shake test). Sit in front of the patient. At the same head
level. Make sure that there is no rheumatoid arthritis or cervical spine problems. Explain
to patient what you are going to do. Hold the head using your hands on either side of the
head. Ask the patient to keep looking at your nose. First make sure that the neck can
move laterally for sufficient range by passive motion. Then, suddenly move the head for
nearly 30 degrees. Usually, the eyeballs suddenly re-fixates to your nose. But in
peripheral lesions, eyeball overshoots, then re-fixates slowly
N Nystagmus. In central – spontaneous vertical or direction changing
Ts Test of skew: Do cover cross-cover test. Vertical misalignment of two eyeballs. i. e. One
eye up & other eye down (central)
Hi N Ts
Peripheral Abnormal Normal: same direction - horizontal Normal Central Normal Direction changing or vertical Abnormal
So in Peripheral only Hi (PHi) ϕ is abnormal!
Additional information
Peripheral has a long incubation period of ~20s compared to central origin nystagmus
Peripheral vision has fatigue (reduced intensity by repetition)
Peripheral nystagmus has habituation (reduce intensity by afternoon)
Peripheral can be dampened by visual fixation – so can use Frenzel glasses to prevent visual
fixation and reduce its intensity
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Central nystagmus is usually from a brain parenchymal lesion e.g. stroke – therefore look for
signs of brainstem or posterior fossa stroke symptoms
9. Pediatric Neurology
a)
Sporadic Other
Tuberous sclerosis 60% AD 40%
Neurofibromatosis 50% AD 50%
Muscular dystrophies 30% XR 70%
b) There is no need to have a vestibular schwanoma to diagnose NF2! Refer to the criteria.
c) Sandifer syndrome has GERD and neuro features (watch a video). Occur when tries to eat
food. Child is mentally impaired, Features – spasmodic torticollis & dystonia, nodding and
rotation of head, neck extension, gurgling, writhing movements of limbs, severe hypotonia,
lasts for 1 to 3 minutes, occur 10 times per day, Diagnosis by history of occurrence while
eating + movement disorder features.
d) Typical absence seizure is seen in childhood absence seizure – Here it is 3 Hz, IQ is normal,
prognosis is good and lasts for 5 to 20s.
Atypical absence – This is seen in juvenile absence seizure. <3Hz, IQ is reduced, poor
prognosis, last <1 minute to several minutes, hyperventilation does not induce it, photic
stimulation rarely induce it.
e)
Equal in male and female children Only males affected
AD AR: WAASP - NF XD XR
HD (HTT gene) DM
(both DM)
NF, TS, VHL, SCA
Tourett’s
JME incomplete
penetrance
Episodic ataxia I,II
neuropathies
Dystonias – DYT1
Alexander, FSH
DRPLA
Emery Dreifuss –
Lamin A&C (LMNA
gene)
APP, PS1, PS2
Familiar FTD+PD
Wilson’s
ARSACS
Ataxia
Tielangiectasia SMA (floppy baby)
PME
Neuroacanthocytosis
(high CK)
Friedereix ataxia
Congenital Muscular
Dystrophy, DYT1
PKAN
LGMD
Aicardi (♀ dies)
Rett
Fragile X
Periventricular
nodular
heterotopia
(Filamin 1)
CMT-X
Incontinenta
pigmenti
DMD (30% sporadic) &
Becker muscular
dystrophy
Fabry
Kennedy (spino-bulbo
muscular atrophy) -
Fasciculations + sensory
neuropathy
ALD (adreno
leukodystrphy)
DYT1, DYT3
Emery Dreifuss -
Emerin
15
10. Stroke
a) Number needed to treat (NNT): (8:6:4:2)
tPA after 3 hours: 8
NASECT: 6
Endovascular treatment: 4%
Anterior temporal lobectomy for temporal lobe epilepsy: 2%
b) Sickle cell disease, Down’s syndrome, Radiation, and Neurofibromatosis
c) Stroke work up in a young patient
Hematologic factors that lead to coagulation:
↓Protein C, S, Antithrombin III, APC (activated (by thrombin) protein C) that inhibits factor V
↑ Fibrinogen (Factor I), Plasminogen (Factor II), factor V Leiden (not inhibited by APC)
Hb electrophoresis (HbS & others), homocystine
Infection: RPR (Rapid plasma reagin), FTA-ABS (Florescent antibody-absorption test)
Immunological: For antiphospholipid antibody syndrome: 1) anti-lupus antibody, 2) beta 2
microglobulin antibody, 3) anti-cardiolipin antibody; ANA
Cardiac: Bubble study for cryptogenic stroke, suspect pulmonary AVM, if everything else fails
d) High INR on warfarin
>10: Hold warfarin + vitamin K 10 mg IV inD5W in 30 minutes
5-10: Hold warfarin + vitamin K 1-2.5 mg IV. Rpt q 12 hours if needed
<5: Omit 1 doze + Continue with a reduced dose
Always investigate the cause: Infection, heart failure, dosing error, liver disease, cancer, poor
nutrition
If actively bleeding: Give fresh frozen plasma. Consult hematology. Consider starting Octaplex
(prothrombin complex concentrate)
11. Telemedicine
a) No NSAIDS in vasculitis, CVST and pseudoxanthoma elasticum
b) GBS-like ascending paralysis is seen in hypophosphatemia
c) Relapse rates in MS
a) MS patient who don’t want to take any medications: 1%
b) MS patient who is taking disease modifying drugs: 0.5%
c) MS during 3rd trimester: 0.2 (very low relapse)
d) MS during postpartum period: 1.2 (high relapse)
Note lactating baby can help reduce this rate slightly. So can advise breast feeding