Neuronal storage disorder with chorea and curvilinear bodies

Mauro Carlo Dal Canto, Isabelle Rapin*, Kinuko Suzuki

*Corresponding author for this work

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

A clinically unique case of ceroid-Iipofuscinosis is described with a juvenile onset, severe chorea that dominates the clinical picture, absence of retinal changes, and uniform intraneuronal inclusions consisting of curvilinear bodies. Cerebral biopsy was performed and ultrastructural and histochemical studies were made. This case differs clinically from the classic late infantile type of nonglycolipid neuronal storage disorder and it differs clinically and ultrastructurally from the classic juvenile type with pigmentary degeneration of the retina. Several atypical variants without visual loss have been reported, but this case appears morphologically distinct from the few cases with a dystonic form recently described ultrastructurally. It supports the notion of nosologic heterogeneity among the nonglycolipid neuronal storage disorders.

Original languageEnglish (US)
Pages (from-to)1026-1032
Number of pages7
JournalNeurology
Volume24
Issue number11
StatePublished - Jan 1 1974

Fingerprint

Ceroid
Chorea
Retina
Biopsy

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Dal Canto, M. C., Rapin, I., & Suzuki, K. (1974). Neuronal storage disorder with chorea and curvilinear bodies. Neurology, 24(11), 1026-1032.
Dal Canto, Mauro Carlo ; Rapin, Isabelle ; Suzuki, Kinuko. / Neuronal storage disorder with chorea and curvilinear bodies. In: Neurology. 1974 ; Vol. 24, No. 11. pp. 1026-1032.
@article{14bc264f673b496f91d749b953da0663,
title = "Neuronal storage disorder with chorea and curvilinear bodies",
abstract = "A clinically unique case of ceroid-Iipofuscinosis is described with a juvenile onset, severe chorea that dominates the clinical picture, absence of retinal changes, and uniform intraneuronal inclusions consisting of curvilinear bodies. Cerebral biopsy was performed and ultrastructural and histochemical studies were made. This case differs clinically from the classic late infantile type of nonglycolipid neuronal storage disorder and it differs clinically and ultrastructurally from the classic juvenile type with pigmentary degeneration of the retina. Several atypical variants without visual loss have been reported, but this case appears morphologically distinct from the few cases with a dystonic form recently described ultrastructurally. It supports the notion of nosologic heterogeneity among the nonglycolipid neuronal storage disorders.",
author = "{Dal Canto}, {Mauro Carlo} and Isabelle Rapin and Kinuko Suzuki",
year = "1974",
month = "1",
day = "1",
language = "English (US)",
volume = "24",
pages = "1026--1032",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "11",

}

Dal Canto, MC, Rapin, I & Suzuki, K 1974, 'Neuronal storage disorder with chorea and curvilinear bodies', Neurology, vol. 24, no. 11, pp. 1026-1032.

Neuronal storage disorder with chorea and curvilinear bodies. / Dal Canto, Mauro Carlo; Rapin, Isabelle; Suzuki, Kinuko.

In: Neurology, Vol. 24, No. 11, 01.01.1974, p. 1026-1032.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Neuronal storage disorder with chorea and curvilinear bodies

AU - Dal Canto, Mauro Carlo

AU - Rapin, Isabelle

AU - Suzuki, Kinuko

PY - 1974/1/1

Y1 - 1974/1/1

N2 - A clinically unique case of ceroid-Iipofuscinosis is described with a juvenile onset, severe chorea that dominates the clinical picture, absence of retinal changes, and uniform intraneuronal inclusions consisting of curvilinear bodies. Cerebral biopsy was performed and ultrastructural and histochemical studies were made. This case differs clinically from the classic late infantile type of nonglycolipid neuronal storage disorder and it differs clinically and ultrastructurally from the classic juvenile type with pigmentary degeneration of the retina. Several atypical variants without visual loss have been reported, but this case appears morphologically distinct from the few cases with a dystonic form recently described ultrastructurally. It supports the notion of nosologic heterogeneity among the nonglycolipid neuronal storage disorders.

AB - A clinically unique case of ceroid-Iipofuscinosis is described with a juvenile onset, severe chorea that dominates the clinical picture, absence of retinal changes, and uniform intraneuronal inclusions consisting of curvilinear bodies. Cerebral biopsy was performed and ultrastructural and histochemical studies were made. This case differs clinically from the classic late infantile type of nonglycolipid neuronal storage disorder and it differs clinically and ultrastructurally from the classic juvenile type with pigmentary degeneration of the retina. Several atypical variants without visual loss have been reported, but this case appears morphologically distinct from the few cases with a dystonic form recently described ultrastructurally. It supports the notion of nosologic heterogeneity among the nonglycolipid neuronal storage disorders.

UR - http://www.scopus.com/inward/record.url?scp=0016289601&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0016289601&partnerID=8YFLogxK

M3 - Article

C2 - 4371401

AN - SCOPUS:0016289601

VL - 24

SP - 1026

EP - 1032

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 11

ER -