RESEARCH: Sporadic OPCA

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Note the last line of this article:

" This case illustrates the great clinicopathological complexity of OPCA and

that not

all forms of sporadic OPCA may be included within MSA. "

Regards,

Pam

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Neurologia 2002 May;17(5):237-243

[Man aged 49 years suffering from progressive clinical picture with palatal

tremor, segmental myoclonus, ataxia, parkinsonism, amyotrophy, pyramidal

signs,

supranuclear ophthalmoplegia and cognitive decline]

Berciano J, Monton F, Maeso M, Ferrer I.

Servicio de Neurologia. Hospital Universitario Marques de Valdecilla (UC).

Santander. Spain.

In this clinicopathological conference we discuss the case of a patient aged

49

years, who developed progressive clinical picture characterized by palatal

tremor (PT), segmental myoclonus, cerebellar ataxia, parkinsonism,

amyotrophy,

pyramidal signs, supranuclear ophthalmoplegia, parkinsonism and cognitive

decline. He died 10 years after onset. There was no family history of

ataxia.

Initially a diagnosis of cerebral Whipple's disease was given, but prolonged

treatment with ampicilin and cloramfenicol did not modify the clinical

course.

Magnetic resonance imaging study showed cerebellar and brainstem atrophy.

Electrophysiological examination revealed neurogenic electromyographic

pattern

and abnormal somatosensory and brainstem evoked potentials. Starting from

symptomatic PT, as the guide sign, a presumptive pathological diagnosis of

sporadic olivopontocerebellar atrophy (OPCA) was established, probably of

multiple system atrophy (MSA) type. Neuropathological study demonstrated

OPCA

with preferential involvement of cerebellum but without glial inclusions.

This

case illustrates the great clinicopathological complexity of OPCA and that

not

all forms of sporadic OPCA may be included within MSA.

PMID: 12031213 [PubMed - as supplied by publisher]