MS and ALS

Question 1

Diagnosis of MS

Answer 1

Must be multifocal (2 lesions) at 2 separate times (must relapse and remit). MRI- multiple inflammatory loci, also r/o chiari malformation. CSF analysis- oligoclonal banding. Evoked potentials- small lesions.

Question 2

Types of MS

Answer 2

Relapsing-Remitting (most common), Secondary progressive, primary progressive

Question 3

Pathologic feature of MS

Answer 3

axonal damage seen by black holes in brain and spinal cord

Question 4

Tx for relapsing-remitting and secondary progressive MS

Answer 4

Long term beta interferon reduces freq of flares.

Question 5

Tx for MS

Answer 5

prednisone for flareups, long term beta interferon, and Natalizumab- reduces brain lesions and relapse rates, but increases risk of progressive multifocal leukoencephalopathy

Question 6

Acute disseminated encephalomyelitis aka

Answer 6

postinfectious encephalomyelitis

Question 7

Dx of devic disease

Answer 7

aka neuromyelitis optica- MRI- involves at least 3 segments of the spinal cord, and specific antibody marker= NMO-IgG

Question 8

symptoms of optic neuritis, acute myelitis=

Answer 8

neuromyelitis optica

Question 9

Tx of devic disease

Answer 9

long term immunosuppressant

Question 10

How to differentiate MS from HTLV-1 associated myelopathy?

Answer 10

Both have similar MRI, EP, and CSF analysis. But HTLV-1 antibodies are present in serum and spinal fluid in HTLV-1 associated myelopathy

Question 11

Slowly progressive weakness and spasticitiy of one of both legs. arms not affected. hyperreflexia, paresthesia. Dx. Autoimmune, inflammatory.

Answer 11

HTLV-1 associated myelopathy

Question 12

What is a spinal cord disorder that can result form HIV?

Answer 12

HIV myelopathy

Question 13

Suspicious of spinal cord lesion. What test is your first go-to?

Answer 13

MRI

Question 14

Dx of HIV myelopathy?

Answer 14

LP to r/o CMV polyneuropathy, MRI to r/o epidural hematoma

Question 15

Deficits in pyramidal and posterior column, polyneuropahy, mental changes, optic neuropathy

Answer 15

Subacute combined degeneration of the spinal cord

Question 16

Subacute combined degeneration of the spinal cord dx, cause, and tx

Answer 16

dx- CBC= pernicious anemia. cause= b12 deficiency. tx= Vitamin B12

Question 17

Giving IV glucose before supplement makes this patient wayyy worse:

Answer 17

Wernicke’s encephalopathy

Question 18

Cause and tx of Wernicke’s encephalopahy

Answer 18

Thiamine deficiency. DO NOT delay tx- thiamine should be given IV

Question 19

Peripheral neuropahy, Korsakoff’s, confusion, ataxia, nystagmus, opthalmoplegia of LR

Answer 19

Wernicke’s encephalopathy

Question 20

2 types of neurogenic bladder

Answer 20

overactive and underactive

Question 21

Dx of progressive bulbar palsy and pseudobulbar palsy

Answer 21

EMG- reduction in number of muscle units under voluntary control. NCS- motor conduction slightly reduced or normal

Question 22

upper vs. lower motor neuron lesion in pseudobulbar palsy and progressive bulbar palsy

Answer 22

progressive bulbar palsy- lower motor neurons affected (CN IV-XII)- flaccid paralysis, fasciculating tongue
Pseudobulbar palsy- upper motor neurons affected- spastic paralysis

Question 23

Spinal muscular atrophy what kind of deficit?

Answer 23

lower motor neuron

Question 24

Family hx, lower limbs affected, absent or nearly absent DTR’s, voice changes, dysphagia, fasciculations of the tongue,

Answer 24

Spinal Muscular atrophy

Question 25

Spinal muscular atrophy and ALS dx

Answer 25

EMG, NCS. EMG should show changes in 3 of the following- bulbar, cervical, thoracic, or lumbosacral regions

Question 26

Degenerative motor neuron diseases

Answer 26

progressive bulbar palsy, pseudobulbar palsy, spinal muscular atrophy, primary lateral sclerosis, amyotrophic lateral sclerosis

Question 27

ALS and primary lateral sclerosis cause what dysfunction?

Answer 27

primary lateral sclerosis- upper motor neuron dysfunction. ALS- mixed

Question 28

“Pseudobulbar affect”

Answer 28

uncontrollable and inappropriate laughing or crying

Question 29

ALS associated with

Answer 29

pseudobulbar affect or parkinsonism

Question 30

Tx for ALS

Answer 30

Riluzole, anticholinergics, baclofen, valium