Clinical and radiological features of juvenile onset olivopontocerebellar atrophy

R. Pratap Chand, J. K J Tharakan, R. L. Koul, S. Dilip Kumar

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Olivopontocerebellar atrophy (OPCA) is uncommon in childhood. We encountered 17 consecutive patients with clinical and radiological features of OPCA with a juvenile onset (mean onset age 8.2 years). These patients were heterogenous on clinical and genetic profiles, but formed three major groups. The largest group consisted of eight patients with OPCA and pigmentary retinal degeneration. The other major groups seen were OPCAs with peripheral neuropathy and OPCA with pyramidal features. Other clinical features seen were extrapyramidal signs and lower cranial nerve palsies. The CT scan showed varying degrees of cerebellar and pontine atrophy. A more precise resolution of the underlying cause of these disorders requires characterization at the genetic and molecular level and awaits further study.

Original languageEnglish
Pages (from-to)152-156
Number of pages5
JournalClinical Neurology and Neurosurgery
Volume98
Issue number2
DOIs
Publication statusPublished - May 1996

Fingerprint

Olivopontocerebellar Atrophies
Cranial Nerve Diseases
Retinal Degeneration
Peripheral Nervous System Diseases
Age of Onset
Atrophy
Molecular Biology

Keywords

  • Olivopontocerebellar atrophy

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery
  • Neurology

Cite this

Clinical and radiological features of juvenile onset olivopontocerebellar atrophy. / Chand, R. Pratap; Tharakan, J. K J; Koul, R. L.; Kumar, S. Dilip.

In: Clinical Neurology and Neurosurgery, Vol. 98, No. 2, 05.1996, p. 152-156.

Research output: Contribution to journalArticle

Chand, R. Pratap ; Tharakan, J. K J ; Koul, R. L. ; Kumar, S. Dilip. / Clinical and radiological features of juvenile onset olivopontocerebellar atrophy. In: Clinical Neurology and Neurosurgery. 1996 ; Vol. 98, No. 2. pp. 152-156.
@article{3d62a15270394ea583835c788672b407,
title = "Clinical and radiological features of juvenile onset olivopontocerebellar atrophy",
abstract = "Olivopontocerebellar atrophy (OPCA) is uncommon in childhood. We encountered 17 consecutive patients with clinical and radiological features of OPCA with a juvenile onset (mean onset age 8.2 years). These patients were heterogenous on clinical and genetic profiles, but formed three major groups. The largest group consisted of eight patients with OPCA and pigmentary retinal degeneration. The other major groups seen were OPCAs with peripheral neuropathy and OPCA with pyramidal features. Other clinical features seen were extrapyramidal signs and lower cranial nerve palsies. The CT scan showed varying degrees of cerebellar and pontine atrophy. A more precise resolution of the underlying cause of these disorders requires characterization at the genetic and molecular level and awaits further study.",
keywords = "Olivopontocerebellar atrophy",
author = "Chand, {R. Pratap} and Tharakan, {J. K J} and Koul, {R. L.} and Kumar, {S. Dilip}",
year = "1996",
month = "5",
doi = "10.1016/0303-8467(96)00012-1",
language = "English",
volume = "98",
pages = "152--156",
journal = "Clinical Neurology and Neurosurgery",
issn = "0303-8467",
publisher = "Elsevier",
number = "2",

}

TY - JOUR

T1 - Clinical and radiological features of juvenile onset olivopontocerebellar atrophy

AU - Chand, R. Pratap

AU - Tharakan, J. K J

AU - Koul, R. L.

AU - Kumar, S. Dilip

PY - 1996/5

Y1 - 1996/5

N2 - Olivopontocerebellar atrophy (OPCA) is uncommon in childhood. We encountered 17 consecutive patients with clinical and radiological features of OPCA with a juvenile onset (mean onset age 8.2 years). These patients were heterogenous on clinical and genetic profiles, but formed three major groups. The largest group consisted of eight patients with OPCA and pigmentary retinal degeneration. The other major groups seen were OPCAs with peripheral neuropathy and OPCA with pyramidal features. Other clinical features seen were extrapyramidal signs and lower cranial nerve palsies. The CT scan showed varying degrees of cerebellar and pontine atrophy. A more precise resolution of the underlying cause of these disorders requires characterization at the genetic and molecular level and awaits further study.

AB - Olivopontocerebellar atrophy (OPCA) is uncommon in childhood. We encountered 17 consecutive patients with clinical and radiological features of OPCA with a juvenile onset (mean onset age 8.2 years). These patients were heterogenous on clinical and genetic profiles, but formed three major groups. The largest group consisted of eight patients with OPCA and pigmentary retinal degeneration. The other major groups seen were OPCAs with peripheral neuropathy and OPCA with pyramidal features. Other clinical features seen were extrapyramidal signs and lower cranial nerve palsies. The CT scan showed varying degrees of cerebellar and pontine atrophy. A more precise resolution of the underlying cause of these disorders requires characterization at the genetic and molecular level and awaits further study.

KW - Olivopontocerebellar atrophy

UR - http://www.scopus.com/inward/record.url?scp=0030152906&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0030152906&partnerID=8YFLogxK

U2 - 10.1016/0303-8467(96)00012-1

DO - 10.1016/0303-8467(96)00012-1

M3 - Article

VL - 98

SP - 152

EP - 156

JO - Clinical Neurology and Neurosurgery

JF - Clinical Neurology and Neurosurgery

SN - 0303-8467

IS - 2

ER -