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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

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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