15.5 (15.5)Neurological disorders other than dementia

Question 1

What is the average prognosis of pts with ALS

Answer 1

2-4 years

Question 2

What are the two major subtypes of ALS? What is the predominant finding in each one early on?

Answer 2

spinal variant - early, asymmetric tetra paresis, late cranial nerve involvement

bulbar variant (20%) - early speech and swallowing, followed by progressive muscle weakness

Question 3

What kind of cognitive impairment can develop in ALS?

Answer 3

Frontotemporal dementia

Question 4

What are the two major positive outcomes identified for the use of NIPPV in patients with ALS?

When should NIPPV should be initiated?

Name 2 objective measures to start NIPPV in ALS patients

Answer 4

improved survival
Improved QOL
Slow rate of resp decline (as measured with FVC)

often started as nocturnal measure in patients with signs of nocturnal hypoventilation (morning HA, increased daytime sleepiness)

objective measures:
- percutaneous nocturnal pulse oximetry
- forced vital capcity (FVC) <65%

Question 5

Thick mucus congestion:
- 2 meds
- 2 non pharm
- 3 mechanical aids

Answer 5

Mucolytic agents (N acetylcysteine)
Nebulized saline + lasix + beta receptor antagonist

Respiratory exercise
Maintain hydration

Mechanical aids (cough assist, high freq chest wall oscillation, home suction device)

Question 6

List 4 medications + 2 medical interventions + 2 alternative ways for managing excessive sialorrhea in ALS

Answer 6

low dose amitriptyline
hyoscine hydrobromide (scopolamine) patch
atropine sulfate
glycopyrollate

botulism injections in salivary glands
salivary gland RT

sage tea
SLP assessment to improve swallowing

Question 7

List medications for rigidity, brady/akinesia

Answer 7

Rotigotine patch
Amantadine
Apomorphine
Midazolam

(RAM)

FS: dopamine agonist

Question 8

List four treatments for spasticity in neurological disease

Answer 8

physiotherapy to maintain full range of motion and avoid contractures

Botox injection (focal spasticity)

nabiximol (synthetic cannaboid)

Muscle relaxants
1. Anticholinergic (cyclobenzaprine)
2. Gaba agonist (baclofen, benzo)
3. Alpha-2 adrenergic agonist (clonidine, tizanidine)

Definition of spasticity: Spasticity is a condition in which muscles stiffen or tighten, preventing normal fluid movement. The muscles remain contracted and resist being stretched, thus affecting movement, speech and gait.

Question 9

What is the first line treatment for muscle cramps in ALS? List one other agent that can be used

Answer 9

levetiracetam - first line
phenytoin, carbamazepine, quinidine sulfate (2nd line)

CSPCP:
First line - baclofen
Treat like muscle spasm

Question 10

What is the pathogenesis of pseudobulbar affect in patients with ALS?

Answer 10

Corticobulbar degeneration

Question 11

What is the primary treatment for pseudobulbar affect? List one other agent that can be used

Answer 11

inappropriate laughter or crying incongruent with mood

Dextromethorphan and quinidine combination - 1st line
SSRI - citalopram
TCA - amitriptyline

Question 12

List motor symptoms of Parkinson’s

Answer 12

Tremor
Rigidity
Akinesia
Postural instability

Question 13

List four symptoms of autonomic instability that may occur in Parkinson’s disease. What disease are these symptoms more common?

Answer 13

orthostatic hypotension*
Sweating *
sialorrhea*
urinary urge incontinence*
constipation*
delayed gastric emptying (gastroparesis)*
erectile dysfunction

More common in multiple system atrophy (MSA)

Question 14

How to manage hallucinations, delusions and psychosis in Parkinson’s?

Answer 14

Keep calm environment

Stop anti-cholinergic meds (e.g. TCA if used for sialorrhea)

Decrease levodopa (dopamine agonist)

Low dose quetiapine (25mg/day)

Question 15

Orthostatic hypotension: List 2 interventions and 2 medications that can be used to manage it

Answer 15

reduction of Levodopa dose
use of compression stockings*
increased Na intake
improved hydration*

meds - fludrocortisone (0.1-0.2mg/d), midodrine (2.5-10mg TID)

Question 16

List 3 drugs for restless leg syndrome in PD

Answer 16

low dose dopamine agonist before bed (rotigotine)
Amantadine
benzodiazepines

valproic acid
gabapentin

FS: like treating tremor?

Question 17

What two Atypical Parkinsonian Disorders (APD) that develop sub-cortical dementia?

What are three features of subcortical dementia?

Answer 17

progressive supranuceal palsy (PSP)
corticobasal degeneration (CBD)

mental slowing
language deficits
impaired memory
apathy
irritability
Depression

Question 18

List four Atypical Parkinsonian Disorders (APD) - display parkinson-like symptoms but do not respond well to antiparkinsonian drugs

Answer 18

dementia with lewy bodies (DLB)
multisystem atrophy (MSA)
progressive supranuclear palsy (PSP)
corticobasal degeneration (CBD)

Question 19

List two treatments for REM sleep behaviour disorder

Answer 19

complex motor manifestations of vivid dreams

clonazepam or melatonin at bedtime

Question 20

A patient at end of life is dealing with rigidity and cannot take his PO dopamine agonist, he does not have IV access - what are two alternatives

Answer 20

rotigotine patch
apomorphine sc
midazolam sc

(RAM)

Question 21

What is the pathogenesis of Multiple Sclerosis?

Answer 21

Immune-mediated inflammatory demyelinating disease of the central nervous system

Question 22

List the two major clinical patterns or phenotypes of MS

Answer 22

a. Relapsing-remitting
b. Primary progressive

Question 23

List 5 presenting symptoms of Multiple Sclerosis (MS).

Answer 23

a. Optic neuritis
b. Paraparesis / paraplegia*
c. Paresthesia in extremities*
d. Lhermitte’s sign (electric shock-like sensation that occurs on flexion of the neck)*
e. Vertigo
f. Bowel dysfunction – constipation*
g. Bladder dysfunction – urinary urgency / incontinence*
h. Sexual dysfunction
i. Fatigue
j. Uhthoff’s phenomenon – symptoms worsen with heat

FS - optic neuritis + lhermitte + weakness, numbness, bladder and bowel changes (similar to cord compression in some ways….)

Question 24

List 5 signs (i.e. physical exam findings) of Multiple Sclerosis.

Answer 24

A. Relative afferent pupillary defect (RAPD)*
B. Lhermitte’s sign*
C. Hyperreflexia*
d. Babinski sign (stimulation of the lateral plantar aspect of the foot leads to dorsiflexion (upward movement) of the big toe)*
e. Spasticity*
f. Gait disturbance*
g. Ataxia*
h. Hemisensory loss

FS:
Inspection - RAPD
Tone - spasticity
Power - gait disturbance
Reflex - hyper reflex
Sensory - hemisensory loss

Other - lhermitte, positive babinski

Question 25

List two investigations required for the diagnosis of MS.

Answer 25

a. MRI brain and spine – to visualise plaque / lesions
b. Lumbar pucture – to check for oligoclonal IgG bands
c. Visual-evoked potentials
d. Optical coherence tomography

Question 26

List two medications to treat trigeminal neuralgia in a patient with MS.

Answer 26

Carbamazepine/Oxcarbazepine (1st line)

• Gaba or lyrica
• Baclofen
• Lamotrigine

Misoprostol