A man aged 58 developed severe left groin pain while walking, eased by flexing the limb, with associated low backache and decreased walking speed. He then noticed deadness and increased sensitivity of his right little finger. He was thought to have a cervical spondylotic radiculomyelopathy, for which a decompressive cervical laminectomy was performed at another hospital in 1985. The clinical and radiological findings at that time are not known. The operation was not helpful and his walking continued to deteriorate. By 1993 his condition had progressively worsened resulting in weakness of his right arm and both legs, sensory disturbance in his hands and in the lower half of his body, and occasional urge incontinence. His balance was poor, especially in the dark, and he was unable to stand without the aid of a stick. There was no family history of polyneuropathy.Examination showed restricted neck and low back movements. There was profound wasting of the forearms and small hand muscles, worse on the right. His upper limbs were very weak, more so distally, with moderate predominantly proximal asymmetric weakness of the lower limbs. All tendon reflexes were absent but the plantar responses were extensor. The peripheral nerves felt slightly thickened. There was hypersensitivity of the skin in both little fingers and medial forearms and sensory loss from the umbilicus downwards in a patchy distribution. Joint position sense was impaired in the feet and vibration could only be detected above the shoulders. He was unable to stand without support but he could take a few steps with the aid of a stick. Romberg's test was strongly positive.
The clinical and electrophysiological findings in 22 patients with chronic trigeminal sensory neuropathy are described. The main clinical feature was slowly evolving unilateral or bilateral facial numbness sometimes associated with pain and paraesthesiae and commonly with disturbed taste. Nine patients had either systemic sclerosis or mixed connective tissue disease. Of the 13 other patients, 9 had either organ or nonorgan specific serum autoantibodies. Blink reflex latencies were recorded in 17 patients, the commonest abnormality being an 'afferent' defect with modest prolongation of latency. Trigeminal sensory evoked responses were recorded in 14 cases, 6 showing mild prolongation of latencies. It is suggested that the lesion in this type of trigeminal neuropathy is in the trigeminal ganglion or in the proximal part of the main trigeminal divisions. This conclusion is supported by limited pathological data.
The authors recruited 19 nonambulant patients with Guillain-Barré syndrome into a pilot, double-blind, randomized, placebo-controlled safety trial of interferon beta 1a (IFN[beta]-1a) (Rebif). Participants received IFN[beta]-1a or placebo subcutaneously three times weekly, 22 microg for the first week and then 44 microg for up to 24 weeks, in addition to IV immunoglobulin (IVIg). IFN[beta] did not have any unexpected interaction with IVIg and there was no significant difference in rate of improvement.
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