Guillain-Barré Syndrome in a Patient with Hereditary Spastic Paraparesis

Autor:	Asmahan Al-Shubaili, Jassim Y. Alhashel, T. Anim, Ashraf Montasser
Rok vydání:	2004
Předmět:	Pediatrics medicine.medical_specialty Henoch-Schonlein purpura Guillain-Barre syndrome immune system diseases business.industry Medicine Spastic paraparesis General Medicine business medicine.disease nervous system diseases Surgery
Zdroj:	Medical Principles and Practice. 13:369-371
ISSN:	1423-0151 1011-7571
DOI:	10.1159/000080476
Popis:	Objective: To report the unusual occurrence of Guillain-Barré syndrome (GBS) in a case of hereditary spastic paraparesis (HSP) and describe its effect on the preexisting clinical picture as well as the importance of early recognition and treatment. Clinical Presentation and Intervention: A 41-year-old man known to have HSP developed an acute and rapid deterioration of muscle power associated with paresthesia in both upper and lower limbs. Clinical examination revealed flaccid quadriparesis with areflexia. Nerve conduction studies were suggestive of demyelinating polyneuropathy and CSF analysis showed increased protein but no cells. The diagnosis of GBS was made in addition to HSP. The patient received intravenous immunoglobulins 20 mg/kg body weight/day for 5 days. He responded very well to the treatment clinically and neurophysiologically and thereafter, he reverted to the preexisting spastic picture of HSP. Conclusion: The peripheral demyelination with GBS was severe enough to obscure the preexisting clinical picture, but fortunately it responded very well to treatment. The clinical associations with HSP are widely variable, therefore it is very important to recognize acquired treatable causes of weakness in such patients in order to prevent an increase in disability.
Databáze:	OpenAIRE
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