Neurosurgery notes/Functional/Movement disorders/Secondary parkinsonism

Secondary parkinsonism

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Parent item
Movement disorders
Sub-item
Progressive supranuclear palsy

General information

  • Aka
    • Parkinson-like conditions
    • “Parkinson plus” syndromes
    • Parkinsonian disorders

Diseases

  • Postencephalitic parkinsonism
    • Followed an epidemic of encephalitis lethargica (von Economo disease) in the 1920s, victims are no longer living.
    • Distinguishing features: oculogyric crisis, tremor involves not only extremities but also trunk and head, asymmetrical, no Lewy bodies
  • Multiple system atrophy (Shy-Drager syndrome)
    • Autonomic dysfunction
    • Progresses much faster than primary Parkinsons's disease
    • MSA-Cerebellar type: Olivopontocerebellar degeneration (OPC)
      • Progressive ataxia
      • MRI: pontine flattening and cerebellar atrophy
    • MSA-Parkinsonian type: Striato-nigral degeneration (SND)
      • More aggressive than parkinsonism
    • Degeneration of preganglionic lateral horn neurons of thoracic spinal cord.
      • Unlike IPA, most do not respond to dopa therapy.
    • NB: classic IPA may eventually produce orthostatic hypotension from inactivity or as a result of progressive autonomic failure.
  • Corticobasal Degeneration (CBD)
    • Progressive neurodegenerative disease of cerebral cortex and the basal ganglia.
    • Symptoms
      • Problems with movement, speech, memory, and swallowing can gradually worsen.
      • Cortical Sensory Loss
        • Damage in the cerebral cortex can lead to loss or impairment of sensory input, which can facilitate hallucinations.
      • Apraxia
        • A motor disorder caused by brain damage of the posterior parietal cortex or corpus callosum.
        • It causes difficulty with motor planning to perform tasks or movement.
  • Drug induced: includes:
    • Prescription drugs (elderly females seem more susceptible)
      • Antipsychotics (AKA neuroleptics)
        • Haloperidol (Haldol®) which works by blocking postsynaptic dopamine receptors
      • Phenothiazine antiemetics
        • Prochlorperazine (Compazine®)
        • Metoclopramide (Reglan®)
        • Reserpine
    • MPTP (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine)
      • A commercially available chemical intermediate which is also a by-product of the synthesis of MPPP (a meperidine analog) that was synthesized and self-injected by a graduate student,21 and later produced by illicit drug manufacturers to be sold as “synthetic heroin” and unwittingly injected by some IV drug abusers in northern California in 1983 (there is also a case report of a chemist who worked with MPTP who developed parkinsonism).
      • MPTP was subsequently discovered to be a potent neurotoxin for dopaminergic neurons (with continued toxic effects that persisted for years).
      • As a rule, the response to levodopa is dramatic, but short-lived with frequent side effects.
      • In contrast to classic IPA, the locus coeruleus and dorsal motor vagus nucleus were essentially normal, and the symptoms differ slightly
    • Methylenedioxymethamphetamine (MDMA)
      • AKA “ecstasy” (on the street),
      • Unproven
      • May hasten the onset of Parkinsonism (a study demonstrating a link had to be withdrawn because of a mislabelling of drugs)
  • Toxic
    • Poisoning with
      • Carbon monoxide: symmetric low densities in the globus pallidus on CT
      • Manganese: may be seen in miners, welders, and pyrotechnics workers. Manganese is excreted by the liver, ∴ people with hepatic insufficiency are more susceptible.
    • Imaging: symmetrical high signal abnormalities on T1WI primarily in the globus pallidus with essentially no findings on T2WI or GRASS (almost pathognomonic)
  • Ischemic (lacunes in basal ganglia)
    • Produces so-called arteriosclerotic parkinsonism AKA vascular parkinsonism
    • “Lower-half” parkinsonism (gait disturbance predominates).
    • Also causes pseudobulbar deficits, emotional lability. Tremor is rare
  • Posttraumatic
    • Parkinsonian symptoms may occur in chronic traumatic encephalopathy, see dementia pugilistica. There are usually other features not normally present in IPA (e.g. cerebellar findings)
  • Normal pressure hydrocephalus (NPH)
    • Urinary incontinence
    • Ataxia
    • Cognitive deficits
  • Neoplasm in the region of the substantia nigra
  • Riley-Day (familial dysautonomia)
  • Parkinson-dementia complex of Guam
    • Classic IPA + amyotrophic lateral sclerosis (ALS).
    • Pathologically has features of parkinsonism and Alzheimer’s disease but no Lewy bodies nor senile plaques
  • Huntington’s disease (HD)
    • Whereas adults typically show chorea, when HD manifests in a young person it may resemble IPA
  • (Spontaneous) intracranial hypotension
    • May present with findings mimicking IPA

Parkinson’s disease and Parkinson’s plus syndromes

Disease
Presentation
Diagnostic
Other
Treatment
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Symptoms common to all Parkinsonian syndromes:
- Resting tremor
- Rigidity (lead pipe or cog-wheel)
- Bradykinesia
- Postural instability
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Disease-specific symptoms
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Parkinson’s disease
- Starts out asymmetrical
Progressively:
– Hypophonia
– Dementia
– Depression
– Frequently alteration of smell
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Degeneration of compacta substantia nigra + Lewy bodies (alpha-synuclein)
1. L-Dopa (long-term use leads to dyskinesias)
2. L-Dopa + Carbidopa (Sinemet: peripheral dopa decarboxylase inhibitor with less side effects) used primarily for freezing episodes
3. Dopamine agonists (bromocriptine, pergolide, cabergoline): less effective than L-Dopa but with delayed risk of dyskinesias
4. MAO-B inhibitors (selegiline)
5. Surgery (deep brain stimulation): Patients with neuropsychiatric disorders are CONTRA-INDICATED
Prognosis: Usually very good with treatment. Often near-normal life expectancy
Progressive supranuclear palsy (Steele–Richardson–Olszewski syndrome)
- Symmetrical
- Reduced voluntary saccades (especially vertical and downward)
- Poor response to L-DOPA
“Humming-bird” sign on MRI (atrophy of midbrain with preservation of pons)
Tauopathy
1. No treatment
2. Prognosis: death in 7 y (from pneumonia + swallowing difficulties)
Disease
Presentation
Diagnostic
Other
Treatment
Multiple system atrophy or Shy–Drager syndrome or olivopontocerebellar atrophy or striatonigral degeneration
-Autonomic dysfunction (orthostatic hypotension/sphincter disturbances/erectile dysfunction) - Ataxia
- There are two types: Parkinsonian and cerebellar
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Accumulation of alpha-synuclein in glial cells
1. L-DOPAtypically doesnotwork. (droxidopa/fludrocortisone)
2. For systolic blood pressure: increase salt intake/compression stockings
3. For bladder dysfunction: anticholinergics (oxybutynin/tolterodine)
Prognosis: average survival 6 y
Corticobasal degeneration
1. Asymmetrical
2. Alien hand syndrome
3. Apraxia/aphasia
Involves cerebral cortex and basal ganglia
Tauopathy
1. Poor response to L-DOPA
2. Prognosis: death within 8 y
  • Parkinson plus syndromes: progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration.