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Sensory neuropathy as the initial manifestation of primary biliary cirrhosis.

Sir: Primary biliary cirrhosis (PBC) is a chronic progressive disorder characterised by cholestasis, liver destruction and circulating mitochondrial antibodies.' Neurological complications of PBC are unusual. In a previous report a pure sensory neuropathy was described in a patient with PBC.2 This study describes a second case ofpure sensory neuropathy as the initial manifestation of PBC. A 62 year old woman was admitted to hospital with a one year history of progressive numbness and clumsiness in her right hand and unsteadiness of gait. She denied pruritus, or gastrointestinal symptoms. General examination was normal. Neurological examination demonstrated normal muscular strength. Deep tendon reflexes were absent. Position and vibration senses were absent in the right hand and severely decreased in the left hand and legs. Loss of the other sensory modalities was found in the same distribution but to a lesser degree. Pseudoathetotic movements were present in the right hand. Gait was ataxic with a positive Romberg's sign. The rest of the neurological examination was normal. Routine laboratory analyses were normal except for a serum alkaline phosphatase level of 1530 U/I (N < 279) and gamma-glutamyl transpeptidase of 552 U/l (N < 65). There was an elevation of the levels of IgG to 2960 mg/100 ml (N: 600-1800) and IgM to 1560 mg/100 ml (N: 50-150). Rheumatoid factor, anti-DNA, Sm, RNP, Ro and La antibodies were negative. Antimitochondrial antibodies were positive at 1:10, 240. Serum electrophoresis, CSF examination, serum B,2 and folate levels, and Schilling's test were normal. Schirmer's and Rose Bengal tests and a minor salivary gland biopsy were negative. Liver biopsy specimen revealed a reduced number of normal bile ducts surrounded by infiltrates oflymphocytes and the presence of small atypical bile ducts with mild portal fibrosis consistent with stage II PBC.' Indirect immunofluorescence on frozen sections of normal human dorsal root ganglia and peripheral nerve incubated with the serum of the patient (1:500) demonstrated staining of the axons with a fine granular pattern. Nuclear membrane of all cell types was also positive. An identical pattern of staining was obtained with the serum of five patients with PBC without sensory neuropathy and high titres of antimitochondrial antibodies. Serum of ten normal controls was negative. Electrophysiological studies disclosed normal motor nerve conduction velocities and distal latencies. Central and peripheral sensory evoked potentials were absent in the four extremities. Needle EMG did not show denervation. A sural nerve biopsy demonstrated a 50% reduction in the number of myelinated fibres. The density of unmyelinated fibres was normal. There were some clusters of small myelinated fibres indicative of regeneration. Teased preparations of 50 myelinated fibres did not demonstrate acute axonal degeneration or primary demyelination. Fourteen fibres had all the internodes of short length suggesting axonal regeneration. Inflammatory or xanthomatous infiltrates. Immunoglobulin or amyloid deposits or abnormalities in the blood vessels were not found. The patient was discharged on azathioprine and colchicine. Seven months later the neuropathy has progressed while the liver disease remains asymptomatic. The association of a sensory neuropathy with PBC was previously reported in a patient who also presented the neuropathy as the initial manifestation of PBC.2 The sural