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P97 Neurological signs, subclinical parameters and quality of life in haemodialysis patients
Neurophysiol Clin (1990) 20, S, 70s-73s
70S
© Elsevier, Paris
Communications affich6es
Posters
SESSION 10 Neuropathies p6riph6riques (2)
Peripheral neuropathies (2)
P96
P97
CARPAL TUNNEL SYNDROME IN MUCOPOLYSACCHARIDOSES AND RELATED DISORDERS S.M. Alani, E Wraith, Royal Manchester Childrens Hospital, Manchester, (UK)
NEUROLOGICAL QUALITY OF
Carpal tunnel syndrome (CTS) is a recognised complication of many of the mucopolysaceharidoses (MPS) and related disorders. It assumes great importance in those patients with mild variants allowing normal intelligence and better prognosis, as the loss of thumb function in a hand with restricted mobility can be a severe handicap. Little is known of the natural history of CTS in MPS disorders. The age of onset is uncertain and the long term response to surgical treatment unknown. Nerve conduction studies were performed in 14 patients (age 20 months-22 years) with various types of MPS as well as 4 patients with MLIII. Clinical evidence of CTS was present in 14 patients and this consisted of wasting of the thenar muscles as well as impaired sensation over the territory of the median nerve in patients old enough to cooperate with testing. All patients had electrophysiological evidence of CTS. None of the patients had symptoms suggestive of CTS such as nocturnal pain or paraesthesiae and Tinel's sign was persistently negative. No patient volunteered a history of numbness or parasthesiae despite clinical evidence of impaired sensation to cotton wool and pinprick. These findings suggest long-standing disease and possibly a poor response to surgical release. Two patients with MPSIH were treated by bone marrow transplantation at ages 14 months and 2 years 3 months. When examined at 7 years 3 months and 7 years respectively both had marked thenar wasting and electrophysiological evidence of severe bilateral CTS. The relatively avascular nature of the flexor retinaculum may explain why BMT did not prevent or reverse the CTS. The early age of onset of CTS, the apparent lack of typical symptoms and its universal occurence in this group of patients indicate the need for greater awareness of the problem in MPS patients. Early diagnosis and treatment offer the best chance of a good result from surgery.
SIGNS, SUBCLINICAL LIFE IN HAEMODIALYSIS
PARAMETERS PATIENTS.
AND
J. Nihom, CAM. Rozeman, EJ. Jonkman, AW. de Weerd. Westeinde Hospital, The Hague, The Netherlands. In a p r o s p e c t i v e l o n g i t u d i n a l s t u d y p a t i e n t s on ch r o n ic ha e modi a l ys i s w e re a n a l y z e d for e n c e p h a l o p a t h y an d p o ly n e u r o p a t h y . N e urol ogi c a l , n e u r o p s y c h o l o g i c a l a nd n e u r o p h y s i o l o g i c a l p a r a m e t e r s w e re i n v e s t i g a t e d in 29 p a t i e n t s d u r i n g at l e a s t 2 y e a r s . At the e n d of t h i s p e r i o d q u a l i t y of life was a s s e s s e d b y means of a q u e s t i o n n a i r e . EEGp a r a m e t e r s p r o v e d to be more s e n s i t i v e in the d e t e c t i o n of e n e e p h a l o p a t h y t h a n a c o m p u t e r i z e d n e u r o p s y c h o l o g i c a l t e s t b a t t e r y . C e r e b r a l d y s f u n c t i o n o c c u r r e d in 68~ of th e cases a t t he s t a r t of the s t u d y . EMG was i n d i c a t i v e of p o l y n e u r o p a t h y in 76~ of t he p a t i e n t s . In t he follow-up the c h a n g e s in t he v a r i o u s c l i ni c a l n e u r o p h y s i o l o g i c a l p a r a m e t e r s w e re a s s e s s e d a n d r e l a t e d to t he r e s u l t s of the q u a l i t y of life s t u d y .
70S
© Elsevier, Paris
Communications affich6es
Posters
SESSION 10 Neuropathies p6riph6riques (2)
Peripheral neuropathies (2)
P96
P97
CARPAL TUNNEL SYNDROME IN MUCOPOLYSACCHARIDOSES AND RELATED DISORDERS S.M. Alani, E Wraith, Royal Manchester Childrens Hospital, Manchester, (UK)
NEUROLOGICAL QUALITY OF
Carpal tunnel syndrome (CTS) is a recognised complication of many of the mucopolysaceharidoses (MPS) and related disorders. It assumes great importance in those patients with mild variants allowing normal intelligence and better prognosis, as the loss of thumb function in a hand with restricted mobility can be a severe handicap. Little is known of the natural history of CTS in MPS disorders. The age of onset is uncertain and the long term response to surgical treatment unknown. Nerve conduction studies were performed in 14 patients (age 20 months-22 years) with various types of MPS as well as 4 patients with MLIII. Clinical evidence of CTS was present in 14 patients and this consisted of wasting of the thenar muscles as well as impaired sensation over the territory of the median nerve in patients old enough to cooperate with testing. All patients had electrophysiological evidence of CTS. None of the patients had symptoms suggestive of CTS such as nocturnal pain or paraesthesiae and Tinel's sign was persistently negative. No patient volunteered a history of numbness or parasthesiae despite clinical evidence of impaired sensation to cotton wool and pinprick. These findings suggest long-standing disease and possibly a poor response to surgical release. Two patients with MPSIH were treated by bone marrow transplantation at ages 14 months and 2 years 3 months. When examined at 7 years 3 months and 7 years respectively both had marked thenar wasting and electrophysiological evidence of severe bilateral CTS. The relatively avascular nature of the flexor retinaculum may explain why BMT did not prevent or reverse the CTS. The early age of onset of CTS, the apparent lack of typical symptoms and its universal occurence in this group of patients indicate the need for greater awareness of the problem in MPS patients. Early diagnosis and treatment offer the best chance of a good result from surgery.
SIGNS, SUBCLINICAL LIFE IN HAEMODIALYSIS
PARAMETERS PATIENTS.
AND
J. Nihom, CAM. Rozeman, EJ. Jonkman, AW. de Weerd. Westeinde Hospital, The Hague, The Netherlands. In a p r o s p e c t i v e l o n g i t u d i n a l s t u d y p a t i e n t s on ch r o n ic ha e modi a l ys i s w e re a n a l y z e d for e n c e p h a l o p a t h y an d p o ly n e u r o p a t h y . N e urol ogi c a l , n e u r o p s y c h o l o g i c a l a nd n e u r o p h y s i o l o g i c a l p a r a m e t e r s w e re i n v e s t i g a t e d in 29 p a t i e n t s d u r i n g at l e a s t 2 y e a r s . At the e n d of t h i s p e r i o d q u a l i t y of life was a s s e s s e d b y means of a q u e s t i o n n a i r e . EEGp a r a m e t e r s p r o v e d to be more s e n s i t i v e in the d e t e c t i o n of e n e e p h a l o p a t h y t h a n a c o m p u t e r i z e d n e u r o p s y c h o l o g i c a l t e s t b a t t e r y . C e r e b r a l d y s f u n c t i o n o c c u r r e d in 68~ of th e cases a t t he s t a r t of the s t u d y . EMG was i n d i c a t i v e of p o l y n e u r o p a t h y in 76~ of t he p a t i e n t s . In t he follow-up the c h a n g e s in t he v a r i o u s c l i ni c a l n e u r o p h y s i o l o g i c a l p a r a m e t e r s w e re a s s e s s e d a n d r e l a t e d to t he r e s u l t s of the q u a l i t y of life s t u d y .