Epilepsy + Huntingtons + MS

Question 1

define epilepsy

Answer 1

if patient meets ONE of the following:

1. at least TWO UNPROVOKED seizures occurring greater than 24 hours apart

or

2. one unprovoked seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occuring over the next 10 years

or

3. diagnosis of an epilepsy syndrome

Question 2

what is the most common symptom of medial temporal lobe seizures

Answer 2

epigastric or visceral aura

Question 3

list triggers for seizure

Answer 3

sleep deprivation

etoh/substances

stress

infection

hypoglycemia

hyponatremia

premenstrual period

head injury

Question 4

what electrolyte disturbance can lead to seizures

Answer 4

hyponatremia

Question 5

what is the most common finding in those patients with intractable temporal lobe epilepsy

Answer 5

mesial temporal sclerosis (hippocampal sclerosis)

Question 6

what are the triad of symptoms associated with huntington’s disease

Answer 6

motor disturbance

cognitive impairment

psychiatric symptoms

Question 7

what is the underlying etiology of huntington’s disease

Answer 7

autosomal dominant disorder

caused by expanded trinucleotide repeat CAG in huntingtin gene of chromosome 4

Question 8

what is the average age of death in huntington’s disease

Answer 8

approx. 40

Question 9

what is considered a prodrome of huntington’s disease

Answer 9

depression

Question 10

psychiatric and cognitive abnormalities can predate the onset of motor sx of huntington’s disease by how long

Answer 10

up to 15 years

Question 11

what are the 4 main clusters of psychiatric symptoms in huntington’s disease

Answer 11

1. depression and anxiety
2. drive and executive function impairment (perseveration, compulsions, apathy)
3. irritability and aggression
4. psychosis

apathy in 70% of symptomatic cases

Question 12

what neurotransmitter changes are seen in the brains of those with huntington’s disease

Answer 12

increased dopamine

decreased GABA

decrease ACh

*also neuronal death via NMDA receptor binding and glutamate excitotoxicity

Question 13

what would you expect to see on neuroimaging in huntington’s disease

Answer 13

caudate nucleus affected prior to symptom onset –> caudate nucleus atrophy and enlargement of the frontal horns of the lateral ventricles

Question 14

what would you use to treat chorea in huntington’s disease

Answer 14

tetrabenazine, amantadine or riluzole

Question 15

what medication has been well studied in the management of the psych symptoms of huntington’s disease

Answer 15

risperidone

–> can improve both psych functioning and motor stabilization

Question 16

what is MS

Answer 16

a chronic inflammatory disease of the central nervous system

has assoc neuropsych sx including DEPRESSION

Question 17

what is the lifetime risk of depression in those with MS

Answer 17

50%

Question 18

how does the presentation of depression differ in those with MS

Answer 18

LESS neurovegetative symptoms

fatigue is common

if fam hx of depression, then chance of depression rises from 50% to 80%

Question 19

how does dx of MS affect risk for suicide

Answer 19

increases risk for suicide

Question 20

what is the gold standard approach to MS diagnosis

Answer 20

MRI

–> periventricular plaques + multiple white matter lesions