PC FastFacts Neurology

Question 1

What is leptomeningeal carcinomatosis?

Answer 1

Spread of tumor cells to the leptomeningeal space

Question 2

Which cancers most commonly cause leptomeningeal disease?

Answer 2

Solid tumor: breast, melanoma, small cell lung

Hematological: leukemia, high grade lymphoma

Question 3

2 mechanisms of leptomeningeal spread

Answer 3

1) hematogenous seeding

2) invasion along nerve roots

Question 4

List s/s of leptomeningeal dz. based on location:

1) brain
2) CNs
3) spinal nerves

Answer 4

1) headache, nausea/vomiting, seizure, hydroceph.
2) hearing loss, diplopia, facial numbness/weakness, dysphonia, dysphagia
3) radiculopathy (pain, paresthesia, weakness), bowel/bladder dysfunction

Question 5

How do you diagnose leptomeningeal dz? what are the findings?

Answer 5

1) CSF - high opening pressure, lymphocytic pleocytosis, high protein, low glucose
- Sens 50-70% one sample, 80-90% 3 samples

2) MRI - meningeal enhancement, hydroceph

Question 6

What are the treatments for leptomeningeal disease?

Answer 6

Radiation
Chemotherapy (including intrathecal)
Steroid (more evidence in lymphoid malignancies)

Question 7

List 3 key features of Huntington’s disease

How is it inherited?

Answer 7

1) Movement disorders
2) Psychiatric manifestations
3) Cognitive deficits

Inheritance - autosomal dominant

Question 8

Describe natural history and prognosis of HD

• Onset
• Symptom trajectory
• Prognosis

Answer 8

Onset - in 30’s and 40’s.

Symptoms - Subtle cognitive and motor change prior to diagnosis. Gradually progressive motor and cognitive decline, often needing institutional support.

Prognosis - Onset to death (15-20yrs). Average age 60.

Question 9

What are motor manifestations of HD and how are they treated?

Answer 9

Chorea, dystonia, rigidity, bradykinesia, tremor, myoclonus

Treatment:

1) VMAT 2 (vesicular monoamine type 2) inhibitors
- dopamine depleting agents
- contraindicated in depression
2) antipsychotics

Question 10

What are common psychiatric manifestations in HD?

Answer 10

Depression - significant, high rate of suicide
Agitation - emotional lability, psychosis
Anxiety

(establish routines to avoid anxiety, PRN benzodiazepines)

Question 11

What are common cognitive deficits in HD?

Answer 11

gradual loss of memory and executive function