Follow-up of neurophysiological tests and CT in late-onset cerebellar ataxia and multiple system atrophy

1993 ◽  
Vol 240 (3) ◽  
pp. 168-176 ◽  
Author(s):  
Karl Wessekl ◽  
Georg-Peter Huss ◽  
Hartmut Br�ckmann ◽  
Detlef K�mpf
Keyword(s):  
Multiple System Atrophy ◽  
Cerebellar Ataxia ◽  
Late Onset ◽  
Neurophysiological Tests

Progression of Nigrostriatal Denervation in Cerebellar Multiple System Atrophy: A Prospective Study

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000013172
Author(s):  
Thomas Wirth ◽  
Izzie Jacques NAMER ◽  
Ben Monga ◽  
Caroline BUND ◽  
Andra Valentina Iosif ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Prospective Study ◽  
Diagnostic Criteria ◽  
Cerebellar Ataxia ◽  
Late Onset ◽  
Final Diagnosis ◽  
Binding Ratio ◽  
A Prospective Study ◽  
Principal Criterion

Background and Objectives:Nigro-striatal dopaminergic denervation (NSDD) remains poorly characterized in cerebellar multiple system atrophy (MSA-C). We aimed to study NSDD progression in MSA-C and evaluate the capacity for [123I]-FP-CIT-SPECT and parkinsonism to differentiate MSA-C from idiopathic late-onset cerebellar ataxia (ILOCA).Material and methods:We included 85 patients successively referred for sporadic late-onset cerebellar ataxia (SLOCA). Every six months, SARA, UPDRS-III and SDFS scores were measured, and MSA-C diagnostic criteria were searched for. Striatal/occipital dopaminergic binding ratio was evaluated every year with [123I]-FP-CIT-scintigraphy.Results:After a mean follow-up of 33.8 months, 33 patients had probable MSA-C, 8 possible MSA-C and 44 ILOCA. SARA and UPDRS-III scores worsened faster in the probable MSA-C group (p<0.01) compared to the ILOCA group. Baseline striatal/occipital ratio was lower (2.3 vs 2.97; p<0.01) and more decreasing among probable MSA-C patients (p<0.01). Weighting dysautonomia and parkinsonism and/or NSDD as additional and principal criterion, respectively, in the possible MSA-C diagnostic criteria slightly improved their specificity (81.6% vs 76.9%) and sensitivity (77.8% vs 72.2%) to predict a final diagnosis of probable MSA-C.Conclusions:Rapid symptoms worsening and NSDD existence and progression predict MSA-C among SLOCA patients. Parkinsonism, NSDD and dysautonomia should be considered equivalent for possible MSA-C diagnosis.

Carpal Tunnel Syndrome in Amyotrophic Lateral Sclerosis and Late Onset Cerebellar Ataxia

1996 ◽  
Vol 21 (4) ◽  
pp. 553-558 ◽  
Author(s):  
M. MONDELLI ◽  
P. DELLA PORTA ◽  
A. ZALAFFI ◽  
A. ROSSI
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Carpal Tunnel Syndrome ◽  
Cerebellar Ataxia ◽  
Carpal Tunnel ◽  
Late Onset ◽  
Axonal Neuropathy ◽  
Electrophysiological Findings ◽  
Tunnel Syndrome ◽  
Lateral Sclerosis

We report on clinical and electrophysiological findings and management in nine patients who developed carpal tunnel syndrome during the course of amyotrophic lateral sclerosis and late onset cerebellar ataxia, two neurodegenerative diseases. The patients were treated with surgical decompression (five cases) and local steroid injections (four cases). Only one showed lasting relief of symptoms and significantly improved distal conduction in the median nerve at follow-up after 2 to 3 months. The symptoms and conduction data remained unchanged in three patients who could be followed for more than 1 year. We think that axonal neuropathy plays an important role in the development of carpal tunnel syndrome in these patients and accounts for the failure of the standard treatments.

scholarly journals CSF analysis differentiates multiple-system atrophy from idiopathic late-onset cerebellar ataxia

Neurology ◽  
2006 ◽  
Vol 67 (3) ◽  
pp. 474-479 ◽  
Author(s):  
W. F. Abdo ◽  
B.P.C. van de Warrenburg ◽  
M. Munneke ◽  
W. J.A. van Geel ◽  
B. R. Bloem ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Cerebellar Ataxia ◽  
Late Onset ◽  
Csf Analysis

CANVAS is a common form of late-onset hereditary ataxia

2020 ◽  
pp. 51-52
Author(s):  
Е.П. Нужный ◽  
Н.Ю. Абрамычева ◽  
Е.Г. Воробьева ◽  
Е.О. Иванова ◽  
Ю.А. Шпилюкова ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Clinical Picture ◽  
Autosomal Recessive ◽  
Late Onset ◽  
Repeat Expansion ◽  
Hereditary Ataxia ◽  
Recessive Ataxia ◽  
Autosomal Recessive Ataxia

Синдром CANVAS (мозжечковая атаксия, невропатия и вестибулярная арефлексия) - аутосомно-рецессивная атаксия с поздним дебютом, обусловленная носительством биаллельной экспансии (AAGGG)n во 2-м интроне гена RFC1. До настоящего момента отсутствуют сведения о распространенности данного заболевания в российских семьях. Нами был проведен поиск биаллельной экспансии AAGGG-повторов у 35 российских пациентов с поздней мозжечковой атаксией. Верифицированы 5 пациентов (14,3%) с синдромом CANVAS и характерной клинической картиной. CANVAS (cerebellar ataxia, neuropathy and vestibular areflexia) is a late-onset autosomal recessive ataxia due to biallelic (AAGGG)n repeat expansion in the 2nd intron of the RFC1 gene. There is no information on the CANVAS prevalence in Russian families. We searched for biallelic expansion of AAGGG repeats in 35 Russian patients with late-onset cerebellar ataxia. Five patients (14.3%) with CANVAS syndrome and a characteristic clinical picture were verified.

Late-onset cerebellar ataxia due to Gordon Holmes Syndrome

2020 ◽  
Vol 79 ◽  
pp. e113-e114
Author(s):  
C.-Y. Kok ◽  
T.-Y. Tee ◽  
A. Karim ◽  
H. Leong
Keyword(s):  
Cerebellar Ataxia ◽  
Late Onset

scholarly journals Hydrocephalus presenting as idiopathic aqueductal stenosis with subsequent development of obstructive tumor: report of 2 cases demonstrating the importance of serial imaging

2017 ◽  
Vol 20 (4) ◽  
pp. 329-333 ◽  
Author(s):  
Jarod L. Roland ◽  
Richard L. Price ◽  
Ashwin A. Kamath ◽  
S. Hassan Akbari ◽  
Eric C. Leuthardt ◽  
...  
Keyword(s):  
Late Onset ◽  
Diagnostic Testing ◽  
Subsequent Development ◽  
Mass Effect ◽  
Aqueductal Stenosis ◽  
Pineal Region ◽  
Serial Imaging ◽  
Cerebrospinal Fluid Diversion ◽  
First Case

The authors describe 2 cases of triventricular hydrocephalus initially presenting as aqueductal stenosis that subsequently developed tumors of the pineal and tectal region. The first case resembled late-onset idiopathic aqueductal stenosis on serial imaging. Subsequent imaging revealed a new tumor in the pineal region causing mass effect on the midbrain. The second case presented in a more typical pattern of aqueductal stenosis during infancy. On delayed follow-up imaging, an enlarging tectal mass was discovered. In both cases hydrocephalus was successfully treated by cerebrospinal fluid diversion prior to tumor presentation. The differential diagnoses, diagnostic testing, and treatment course for these unusual cases are discussed. The importance of follow-up MRI in cases of idiopathic aqueductal stenosis is emphasized by these exemplar cases.

Sign in / Sign up

Export Citation Format

Share Document