Multiple System Atrophy

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Multiple system atrophy (MSA) is characterized clinically by a combination of parkinsonian, pyramidal, cerebellar, and autonomic symptoms. In contrast to Parkinson's disease, rest tremor is usually absent, and the findings are relatively symmetric. The autonomic symptoms are disabling and help differentiate MSA from other parkinsonian disorders.

The pathological features include cell loss and gliosis in the striatum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, dorsal vagal nuclei, Purkinje cells of the cerebellum, and Onuf's nucleus of the caudal spinal cord.

Neurochemically, low levels of dopamine in the substantia nigra and striatum have been shown in postmortem studies.

Neuroimaging using magnetic resonance imaging (MRI) often reveals areas of bilateral decrease in signal density in the posterolateral putamen on T2-weighted images. Positron-emission tomography (PET) studies showed reduced striatal and frontal lobe metabolism.

Shy-Drager syndrome. Dysautonomia is the most characteristic clinical feature of Shy-Drager syndrome (SDS). Patients show reduced 18F 6-fluorodopa uptake, indicating nigrostriatal dysfunction.

Manifestations (+) Possible other features (±)

Bradykinesia Ataxia

Rigidity Dementia

Gait disturbance Dysarthria/dysphagia

Dysautonomia Motor neuron disease

Sleep impairment Neuropathy

Putaminal T2 hypointensity Oculomotor deficit Levodopa response Lewy bodies

Striatonigral degeneration. Respiratory dysregulation with laryngeal stridor and sleep apnea are often prominent clinical features in striatonigral degeneration (SND). Decreased D2-receptor density has been found in patients with SND. Vasomotor impairment in SND has been attributed to a selective loss of tyrosine hydroxylase - immunoreactive neurons in the A1 and A2 regions of the medulla oblongata.

Manifestations (+)

Possible other features (±)

Bradykinesia Rigidity

Gait disturbance Dysarthria/dysphagia Putaminal T2 hypointensity Levodopa dyskinesia Lewy bodies

Dysautonomia Dystonia Eyelid apraxia Motor neuron disease Sleep impairment

Olivopontocerebellar atrophy. Cerebellar ataxia is the most frequent presenting symptom in patients with olivopontocerebellar atrophy (OPCA). MRI on T2-weighted images shows pancerebellar and brain stem atrophy, enlarged fourth ventricle and cerebellopontine angle cisterns, and demyelination of transverse pontine fibers.

A reduction in dopamine has been found in 53% of cases in the putamen, 35% in the caudate, and 31% in the nucleus accumbens. Mito-chondrial deoxyribonucleic acid abnormalities may be important in the pathogenesis of OPCA.

Manifestations (+)

Possible other features (±)

Rigidity

Gait disturbance Ataxia

Dysarthria/dysphagia Oculomotor deficit Putaminal T2 hypointensity

Bradykinesia Tremor Dysautonomia Neuropathy Sleep impairment Lewy bodies

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