Questions, NeuroSAE 1

Question 1

HIV distal polyneuropathy - similar to DM neuropathy.

Answer 1

It is the most common complication of HIV regardless of the viral load. Affects 35 % of HIV patients, it is predominantly small fiber unmyelinated neuropathy.
Always check B12, syphilis as they are treatable conditions.

Question 2

Cluster headache treatment

Answer 2

Most efficacious treatment is to sub q sumatriptans.
High flow oxygen at 100 % concentration through face mask with 6 to 12 L per minute.
Oxacarbazepin or carbamazepine are used for cluster tic syndrome.

Question 3

Concussive convulsion

Answer 3

occurs after head trauma. No AED are needed but they require to be on post concussion protocol.
Post concussive seizure- chronic- is after one week of the seizure onset.
If MRI is abnormal after a head trauma then a prophylactic AED FOR 7 DAYS ARE INDICATED.

Question 4

In ALS

Answer 4

Indication for PEG tube placement is decreased vital capacity by less than 50 % and the drop in BMI below 18.5
It will prolong life by 1 to 4 months..

Question 5

Inhalation of N hexan

Answer 5

Causes distal symmetric polyneuropathy.
It is rapidly and irreversible.
In nitrous oxide inhalation it causes more posterior column rather

Question 6

The diagnosis of Chronic traumatic encephalopathy

Answer 6

two or more of cognitive or behavioral disturbance, cerebellar dysfunction, pyramidal or extrapyramidal dysfunction.

Question 7

Kinisogenic dyskenisa

Answer 7

autosomal dominant, more common in male than female. associated with exertion. It lasts from seconds to minutes. responds well to carbamazepine and other AED. Not a seizure

Question 8

Nonkinisogenic dyskensia

Answer 8

It lasts from minutes to hours, it is caffeine or alcohol triggered. It does not respond to AED.

Question 9

Paroxysmal hypnogonic dyskensia

Answer 9

localized to a seizure from the frontal supplementary association area that occurs out of sleep.
It lasts from minutes to 30 minutes.

Question 10

fovatriptans

Answer 10

Are evidence level A to prevent perimenstrual migraine headache.

Question 11

Congenital Myotnic dystrophy

Answer 11

Type 1 is infentile.
It is due to CTG gene expansion in DMPK.
In infants there is hypotonia but no myotonia.
It is associated with anticipation.

Type 2 it is associated with ZNF 9 mutation
FSH is associated with DZ4Z mutation.

Question 12

Arterial thrombosis

Answer 12

Antiphspholipids syndrome.
Lupus anticoagulant
pro thrombin gene mutation
beta 2 glycoprotein.

Question 13

sausac syndrome

Answer 13

It is a small angiopathic autoimmune disease causes CNS symptoms, retinal disease and hearing loss because of the involvement of the retinal artery branch occlusion, inner ear and cns.

Question 14

Menier’s disease

Answer 14

prolonged episodes of vertigo that can last minutes to hours.
It is associated with tinnitus, sensorineural hearing loss and sensation of ear fullness.
It is due to expansion of the endoplymph over the perilymph.
The patient usually prefers to sleep on the healthy side to keep the affected side up.

Question 15

Open lip schezoencephaly

Closed lip schezencephaly

Answer 15

It is a disease of neuronal migration. It tends to cause seizures.
In peri-natal infarction: it causes encephalomalacia and will have intact ventricular wall.

Question 16

Kennedy disease. Xlinked spinomuscular atrophy

Answer 16

It is lower motor neuron disease.
It causes facial/peri-oral fasciculation and limb girdle muscle weakness pattern. Tongue weakness and atrophy, with gynecomastia.
The gene mutation is CAG trinucleotide repeat on androgen, it is X linked.

It is slowly progressive disease that

Question 17

NMS

Answer 17

Occurs from D2 blocking agent. It causes rigidity, hyperthermia, elevated CK and leukocytosis with encephalopathy.
It is causes by atypical neuroleptics.
Treatment is to stop the offending agent, hydration and supportive.
The malignant hyperthermia, it occurs in response to anesthetic agent usually sooner than NMS. They are are due to gene mutation in rayndion.

Question 18

CNS toxoplasmosis

Answer 18

Diagnosis is made by ring enhancing lesions, IgG and no prior prophylaxis with Bactrim.
Improvement is seen in 1 to 2 weeks and imaging improvement is seen in 2-3 weeks
Sulfadiazine and pyrimethamine achieve treatment in 60 to 90 %.
The prophylaxis can stop after CD count > 100 for 6 months.

Question 19

subacute multifocal leukoencephalopathy

Answer 19

JC virus.
T 2 hyper-intense lesions that do not enhance or have mass effect.
It is localized to the white matter.

Question 20

New daily persistent headache.

Answer 20

Acute onset, persistent daily headache for 3 months with features similar to migraine headache.

Question 21

FTD

Answer 21

can present with weight gain.

Question 22

rabies encephalitis

Answer 22

causes headache, behavioral changes, spasms, difficulty swallowing and drooling

Question 23

Post concussion assessment

Answer 23

Physical- physical includes visual tracking, balance and head drop clinical reaction.
Cognitive- Impact test.

Question 24

Tizanidine

Answer 24

good for spinal cord inury spasticity but causes depression and hypotension.

Question 25

Hyperekplexia

Answer 25

This is caused by alpha subnite 1 glycine receptor- glycine is a major inhibitory neurotransmitter.
It causes exaggerated startle that does not habituate with repeated stimuli.
These patients have autosomal dominant inheritance. .

Question 26

spice- marijuana intoxication

Answer 26

It is a cheap analogue of marijuana that is often used.

Question 27

Thyroxine, theophylline., tacroliums cause

Answer 27

Cause postural tremor.

Question 28

Haloperidol

Answer 28

cause resting tremor.

Question 29

Alemtuzumb

Answer 29

it is a monoclonal antibody that is directed against CD52 T cells. it is used as an induction therapy in aggressive MS.
It has the risk of re-emergence of autoimmune disease remotely from the time of treatment.

Question 30

Injectable MS drugs

Answer 30

Interferons.

Glatiremer acetate

Question 31

Oral agents for MS

Answer 31

Fingolamid- sphinogsene 1 phosphatase
It causes bradycardia, macular edema, transaminases, and increases risk of herpetic infection. Must ensure immunity against varicella before starting the medication is a must. there are cases of PML reported with fingolamid.
Periodic skin assessment because of risk of skin cancer.
Ophthalmological evaluation for macular edema.

Teriflonamide: it is a dihydroorate inhibitor that inhibits synthesis of pyramidine in rapidly dividing cells.

Side effects include: elevated transaminases and infection. It also causes diarrhea and hair thining.
It is category X, and is also excreted in seminal fluid therefore males have to have contraception method.

ocrevus- ocrilzumab- it is a monoclonal antibody ragerting CD20

Question 32

Pregnancy and use of disease modifying agents

Answer 32

Interferon to be stopped 2 months before pregnancy
Glatimere acetate is considered safe
fingolamide causes persistent trunks arteriosis and VSD, it should be stopped 2 monthes before conception.
Teriflonamide is catergory x, if woman becomes pregnant, she must undergo elimination with cholestyramine

Question 33

fingolamid

Answer 33

if patient misses medication for 2 weeks needs first dose monitoring

Question 34

fragil X tremor ataxia syndrome.

Answer 34

Fragil X is a trinuclitide repeat on chromosome X. Premution causes a different phenotype in female patients with primary ovrian failure, mixed tremor and mild executive dysfunction. symptoms develop after the age of 50

Question 35

Mobixnol

Answer 35

reduce subjective spasticity but not objective

Question 36

Nitrous oxide

Answer 36

causes B12 deficiency

Question 37

In the treatment of NMP spectrum

Answer 37

Rituximan is the preferred agent. It is a monoclonal antibody against CD20 cells we monitor with B cells CD 19 re-emergence.
In mycophenolate- the lymphocyte count must be less than 1500
In Azathioprine- we must check TMPT.

Question 38

Acute flaccid myelitis

Answer 38

seen in children where is involvement of the anterior horn grey matter.

Pediatrics MS tend to have larger lesion since and involvement of the brain stem and cerebellum.

Question 39

anti-MOG antibodies

Answer 39

are recent discovered in patients with acute demyelinating diseases.

Question 40

Kids with ADEM

Answer 40

are at risk of other auto-immune disorders such as NMDA.

Question 41

Pediatrics transverse myelitis

Answer 41

treat with IV steroids, if it fails consider PLEX.

Question 42

Pediatrics MS

Answer 42

has high volume burden and infra tentorial involvement.

Question 43

Hereditary leukoencephalopathy with spheroids

Answer 43

It is autosomal dominant with frontal lobe symptoms, and extrapyramidal symptoms. CSF1R gene mutation/

Question 44

DRPLA

Answer 44

clinical features are: choreoathetosis, cognitive decline, ataxia and myoclonic seizures. IT IS CAUSED BY TRINEUCLITIES EXPANSION.
IT IS AUTOSOMAL DOMINANT WITH INVOLVEMENT OF THE CAG REPEAT.

Question 45

cerebral autoimmune hereditary leukodystrophy

Answer 45

gene mutation is lamin B1

It has early onset autonomic dysfunction, and cerebellar symptoms.

Question 46

cerebrotendnous xanthomatosis

Answer 46

It is caused be deficiency in mitochondrial 27 hydroxylase.
Cataract, cognitive decline and xanthomas
It is treatable with chenodeoxy acid.
It is a lipid storage disease.

Question 47

fabry disease

Answer 47

it is x linked
caused by deficiency of alpha galactosidase.
It causes angiokeratoma, chronic acral paresthesia and renal failure.