Recurrent Guillain Barre' Syndrome: a clinical, electrophysiological and morphological study.

Of the 220 patients of acute idiopathic demyelinating polyneuritis (AIDP/GBS) seen over a seven year period, 15 patients (M:F:11:4) had a relapsing course (6.8%). Their ages ranged from 8 yrs to 70 yrs. They had 36 episodes at a variable interval of 3 months to 25 yrs. Relapse rate varied from one to four. Antecedent events were noted during 16 episodes in 9 patients but the triggering factors were varied. Clinical features of individual episodes were similar to the acute monophasic illness, although they differed inseverity from one episode to the other. Autonomic disturbances were rare. Albuminocytological dissociation was observed during 19 of the 24 episodes. Electrophysiological abnormalities were observed during 19 of the 24 episodes. Electrophysiological abnormalities were present in all and were comparable with patients of non-recurrent illness. Sural nerve biopsy in 3 patients showed evidence of demyelination, remyelination, Wallerian degeneration and myelin breakdown but none had features of inflammation. With the exception of one death, functional recovery was complete in the majority of patients, irrespective of the type of therapeutic intervention. Acute onset, frequent facial involvement, brief clinical course, near complete recovery and very long asymptomatic periods may distinguish these patients of acute relapsing demyelinating polyneuropathy (ARDP) from chronic relapsing demyelinating polyneuropathy. Relapses in GBS are however unpredictable and recurrent GBS is indistinguishable clinically, electrophysiologically and morphologically from the more frequently seen non-recurrent form of monophasic GB Syndrome. A biochemical or immunological marker may help in this distinction.

Neurological complications due to Semple-type antirabies vaccine. Clinical and therapeutic aspects.

Thirty nine patients with neuroparalytic accidents due to the use of Semple-type antirabies vaccine were studied. The mean age of the patients was 25.8 +/- 13.2 years. The suspected source of infection was the bite of a dog in 36 (92.3%) cases. The mean interval between the first dose of ARV and the onset of neurological deficits was 14.4 +/- 8.7 days. The number of doses was 7 or less in 28 (71.8%) and more than 7 in 11 (28.2%) cases. With regard to neurological deficits, 5 (12.8%) had encephalopathy, 1 (2.6%) had encephalomyeloradiculopathy, 12 (30.7%) had cervical myeloradiculopathy, 4 (10.3%) had dorsolumbar myeloradiculopathy and 17 (43.6%) had polyradiculopathy. Lumbar cerebrospinal fluid analysis was done in 31 (79.5%) cases and was abnormal in 15 (48.4%), in the form of pleocytosis or raised protein or both. Electroencephalogram was done in 24 (61.5) cases and was abnormal in 7 (29.2%); in 6 (85.7%) of theme the abnormalities were subclinical. Electroneuromyography was done in 15 (38.5%) patients and was abnormal in 13 (72.2%). Visual evoked potentials were studied in 11 (28.2%) cases and were abnormal in 2 (18.2%). Thirty six (92.3%) cases received steroids and 25 (64.1%) received cyclophosphamide in addition. The therapeutic results were better in those who received cyclophosphamide. Three patients died; One died due to respiratory failure and two due to unrelated causes while on respirator. The latter two were autopsied, and findings in the brain were unremarkable.