Non-PD Movement Disorders

Question 1

Progressive Supranuclear Palsy (PSP)

Answer 1

Tauopathy
Hallmark: Gait imbalance with falls
- Restricted Vertical Gaze (predominantly downward gaze on examination)
- Retrocollis: Involuntary neck hyperextension
- Square wave jerks, Impaired optokinetic nystagmus
- MRI: Hummingbird Sign

Disease onset ~1.5 years from symptom onset

Question 2

Hummingbird Sign

Answer 2

PSP

Atrophy of the MIDBRAIN

Question 3

Multiple System Atrophy (MSA)

Answer 3

Alpha-Synuclein
Early age of onset
Hallmark: Autonomic dysfunction with Orthostatic Hypotension without compensatory mechanisms, Urinary incontinence*, Impotence
- Antecollis: Involuntary Neck Flexion
- Prominent Ataxia
Pathology: Cell loss & gliosis in the Striatum & Substantia Nigra. Putamen is the most affected.
MRI: Hot-Cross Buns Signs, Hypointensity of the Putamen on T2 or hyperintense slit-like rim around the putamen.

Question 4

MSA-A

Answer 4

MSA with significant autonomic dysfunction (Orthostatic Hypotension)
Shy-Drager Syndrome
Oppenheimer’s Syndrome
Pathology: Neuronal loss & atrophy in the intermediolateral cell columns of the spinal cord

Question 5

MSA-P

Answer 5

MSA with significant Parkinsonism.
Striatonigral Degeneration

Akinetic Rigid Syndrome
Akinesia, Rigidity, Dysphonia, Postural Instability, Autonomic dysfunction.
Anterocollis
Stridor 2/2 Laryngeal Abductor Paralysis

Question 6

MSA-C

Answer 6

MSA with cerebellar ataxia
Olivopontocerebellar Atrophy.
Pathology: Neuronal loss and atrophy in the Pons, Medullary Olives, Cerebellum

Question 7

Hot-Cross-Buns Signs

Answer 7

MSA
Transverse and vertical hyperintensity in the PONS due to loss of pontine neurons and pontocerebellar tracts with intact corticospinal tracts

Question 8

What causes urinary incontinence in MSA?

Answer 8

Due to involvement of the group of Anterior Horn Cells in the sacral cord - Onuf’s Nucleus

Question 9

Corticobasal Syndrome (CBS)

Answer 9

Tauopathy
Hallmark: The presence of focal limb rigidity +/- dystonia, cortical myoclonus, cortical sensory loss*
- Cortical Sensory Loss: Asterognosis, Agraphesthesia, Loss of 2-point discrimination
- Frontal/Subcortical pattern of cognitive dysfunction, Apraxia, Alien Limb Phenomenon
Due to corticobasal ganglionic degeneration (CBD) - Deposition of phosphorylated tau and neuronal degeneration in the pre- and post-central cortical areas, ganglia, thalamus, substantia nigra

Question 10

How does MSA differ from PD?

Answer 10

Unexplained falls in early disease
Early appearance of autonomic symptoms
Rapid progression of Parkinsonian disability
Lack/Unsustained response to Levodopa 
Symmetric presentation
Minimal/Resting tremor

Question 11

Autosomal Dominant.
Trinucleotide CAG repeat in the Huntingtin gene in Chromosome 4

Path: Atrophy of the Caudate, Loss of medium spiny striatal neurons accompanied by gliosis

Biochemically: Decreased gamma-aminobutyric acid, Enkephalins, Substance P

Answer 11

Huntington’s Disease

Question 12

Treatment of Chorea in HD

Answer 12

Dopamine Receptor-blocking Agents: Haloperidol, Risperidone, Clozapine, Quetiapine, Reserpine, Tetrabenazine
ASM: VPA, Carbamezapine
Clonazepam, Amantadine, Tetrabenazine