Alexander's Disease with Rosenthal Fibre Formation in an Adult

Overview

Journal J Neurol Neurosurg Psychiatry

Publisher BMJ Publishing Group

Specialties Neurology
Neurosurgery
Psychiatry

Date 1984 Apr 1

PMID 6726266

Citations 10

Authors

T J Walls

R A Jones

N Cartlidge

M Saunders

Affiliations

Soon will be listed here.

Abstract

The case of a woman who had a mild mental and physical handicap all her life is described. At the age of 39 years she began a progressive deterioration which terminated with her death after a year. CT scanning revealed basal ganglia calcification and at necropsy there was evidence of widespread generalised myelin loss with extensive Rosenthal fibre formation. The hypothesis that this may be an adult form of Alexander's disease is discussed.

Citing Articles

Encephalopathies with intracranial calcification in children: clinical and genetic characterization.

Tonduti D, Panteghini C, Pichiecchio A, Decio A, Carecchio M, Reale C Orphanet J Rare Dis. 2018; 13(1):135.

PMID: 30111349 PMC: 6094574. DOI: 10.1186/s13023-018-0854-y.

Late-onset Alexander disease with a V87L mutation in glial fibrillary acidic protein (GFAP) and calcifying lesions in the sub-cortex and cortex.

Suzuki H, Yoshida T, Kitada M, Ichihashi J, Sasayama H, Nishikawa Y J Neurol. 2011; 259(3):457-61.

PMID: 21822933 DOI: 10.1007/s00415-011-6201-z.

Adult-onset Alexander disease : report on a family.

Balbi P, Seri M, Ceccherini I, Uggetti C, Casale R, Fundaro C J Neurol. 2007; 255(1):24-30.

PMID: 18004641 DOI: 10.1007/s00415-007-0654-0.

Alexander disease: diagnosis with MR imaging.

van der Knaap M, Naidu S, Breiter S, Blaser S, Stroink H, Springer S AJNR Am J Neuroradiol. 2001; 22(3):541-52.

PMID: 11237983 PMC: 7976831.

A familial disorder associated with palatal myoclonus, other brainstem signs, tetraparesis, ataxia and Rosenthal fibre formation.

Howard R, Greenwood R, Gawler J, Scaravilli F, Marsden C, Harding A J Neurol Neurosurg Psychiatry. 1993; 56(9):977-81.

PMID: 8410038 PMC: 489732. DOI: 10.1136/jnnp.56.9.977.

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