Dysautonomic features in patients with Cuban type 2 spinocerebellar ataxia

被引:14
作者
Sánchez-Cruz, G [1 ]
Velázquez-Pérez, L [1 ]
Gómez-Peña, L [1 ]
Martínez-Góngora, E [1 ]
Castellano-Sánchez, G [1 ]
Santos-Falcón, N [1 ]
机构
[1] CIRAH, Holguin 80100, Cuba
关键词
Cuban ataxia; dysautonomia; hereditary ataxia; hereditary spinocerebellar degeneration. neurovegetative features; olivo-ponto-cerebellar atrophy; type 2 spinocerebellar ataxia; visceral features;
D O I
10.33588/rn.3305.2001244
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Introduction. Dysautonomic features are the clinical signs and symptoms derived from anomalous functioning of the sympathetic or parasympathetic nervous systems in either the peripheral or central parts. Objective. To identify the clinical features related to dysautonomia in a patient with spinocerebellar ataxia with severe functional disability. Patients and methods. We studied a series of cases, including 21 patients with severe disability (confirmed to their bed or wheelchair. unable to walk and totally or partly dependent on other people for essential everyday activities). The patients and their families were closely questioned, and full clinical examination included a test for orthostasia. Results. All patients had some signs of peripheral dysautonomia: all had vasomotor disorders (orthostasia, distal pallor and coldness, Raynaud's phenomenon, etc.). 95.2% (constipation, urinary and rectal incontinence, polachuria, palpitations, tachycardia at rest, etc.), exocrine gland disorders in 71.4% (increased lachrymation, reduced sweating, increased or reduced salivation), 87.5% peripheral tissue nutrition disorders. Similarly, in all patients studied there was evidence of central dysautonomic disorder, with a syndrome of cachexia with bulimia, sleep disorders together with dysregulation of thirst and body temperature. Conclusions. In patients with type 2 hereditary spinocerebellar ataxia with severe disability there was involvement of the peripheral and central nervous system regulating autonomic function.
引用
收藏
页码:428 / 433
页数:6
相关论文
共 75 条
[1]  
ADAMS RD, 1982, PRINCIPIOS NEUROLOGI, V1, P381
[2]   Autonomic response to change of posture among normal and mild-hypertensive adults: Investigation by time-dependent spectral analysis [J].
Akselrod, S ;
Oz, O ;
Greenberg, M ;
Keselbrener, L .
JOURNAL OF THE AUTONOMIC NERVOUS SYSTEM, 1997, 64 (01) :33-43
[3]   Evaluation of congenital dysautonomia other than Riley-Day syndrome [J].
Alvarez, E ;
Ferrer, T ;
PerezConde, C ;
LopezTerradas, JM ;
PerezJimenez, A ;
Ramos, MJ .
NEUROPEDIATRICS, 1996, 27 (01) :26-31
[4]  
BANNISTER R, 1993, AUTONOMIC DISORDERS, P517
[5]   CENTRAL AUTONOMIC DISORDERS [J].
BENARROCH, EE ;
CHANG, FLF .
JOURNAL OF CLINICAL NEUROPHYSIOLOGY, 1993, 10 (01) :39-50
[6]  
BHATT MH, 1992, TXB INTERNAL MED, P2176
[7]   Cardiovascular parameters: Sensitivity to detect autonomic dysfunction and influence of age and sex in normal subjects [J].
Braune, S ;
Auer, A ;
SchulteMonting, J ;
Schwerbrock, S ;
Lucking, CH .
CLINICAL AUTONOMIC RESEARCH, 1996, 6 (01) :3-15
[8]  
Brown S, 1892, Brain, V15, P250
[9]  
CHOKROVERTY S, 1984, OLIVOPONTOCEREBELLAR, P105
[10]   Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion [J].
David, G ;
Abbas, N ;
Stevanin, G ;
Durr, A ;
Yvert, G ;
Cancel, G ;
Weber, C ;
Imbert, G ;
Saudou, F ;
Antoniou, E ;
Drabkin, H ;
Gemmill, R ;
Giunti, P ;
Benomar, A ;
Wood, N ;
Ruberg, M ;
Agid, Y ;
Mandel, JL ;
Brice, A .
NATURE GENETICS, 1997, 17 (01) :65-70