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11/1370. Successful treatment of IgM paraproteinaemic neuropathy with fludarabine.

    OBJECTIVES: To evaluate the response of four patients with IgM paraproteinaemic neuropathy to a novel therapy-pulsed intravenous fludarabine. BACKGROUND: The peripheral neuropathy associated with IgM paraproteinaemia usually runs a chronic, slowly progressive course which may eventually cause severe disability. Treatment with conventional immunosuppressive regimens has been unsatisfactory. Fludarabine is a novel purine analogue which has recently been shown to be effective in low grade lymphoid malignancies. methods: Four patients were treated with IgM paraproteinaemic neuropathy with intravenous pulses of fludarabine. Two of the four patients had antibodies to MAG and characteristic widely spaced myelin on nerve biopsy and a third had characteristic widely spaced myelin only. The fourth had an endoneurial lymphocytic infiltrate on nerve biopsy and a diagnosis of Waldenstrom's macroglobulinaemia. RESULTS: In all cases subjective and objective clinical improvement occurred associated with a significant fall in the IgM paraprotein concentration in three cases. Neurophysiological parameters improved in the three patients examined. The treatment was well tolerated. All patients developed mild, reversible lymphopenia and 50% mild generalised myelosuppression, but there were no febrile episodes. CONCLUSION: Fludarabine should be considered as a possible treatment for patients with IgM MGUS paraproteinaemic neuropathy.
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keywords = neuropathy
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12/1370. Extremely acute phenytoin-induced peripheral neuropathy.

    PURPOSE: Peripheral neuropathy is a rare adverse effect associated with phenytoin (PHT), and it usually occurs after the prolonged use of PHT. Acute PHT-induced peripheral neuropathy is extremely rare. methods: An 18-year-old girl was admitted for the control of epilepsy. Just a few hours after the administration of PHT, she complained of distal lower-extremity paresthesia in a stocking distribution and motor weakness: the achilles tendon reflex was absent. RESULTS: Electrophysiological studies revealed slightly reduced sensory-conduction velocity and mild prolongation of distal latency in the lower extremities. After the discontinuation of PHT, these symptoms disappeared gradually, and sensory-conduction velocity and distal latency became normal. CONCLUSIONS: Although it has been reported that peripheral neuropathy occurred after treatment with PHT for a week, there has been no report of a patient such as ours, who developed peripheral neuropathy just a few hours after the initial administration of PHT. The underlying mechanism remains unknown; however, we should pay attention to such extremely acute peripheral neuropathy when using PHT.
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ranking = 1.125
keywords = neuropathy
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13/1370. November 1998--70 year old woman with SLE, paraproteinemia and polyneuropathy.

    A 70 year old woman developed a sensory-motor polyneuropathy 12 years after almost continuous treatment with hydrochloroquine for SLE. sural nerve biopsy disclosed abundant lipid inclusions in schwann cells and vessels against a background of axonal degeneration. After cessation of therapy the patient improved moderately. This case underscores the usefulness of nerve biopsy in the study of management of patients with peripheral neuropathy associated with systemic illnesses.
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ranking = 0.75
keywords = neuropathy
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14/1370. polyradiculoneuropathy revealing a solitary plasmacytoma of the ilium. A new case-report.

    Neurological manifestations are uncommon in myeloma patients, and subacute polyradiculoneuropathy as the inaugural manifestations of solitary plasmacytoma of bone is exceedingly rare. We report the case of a 52-year-old man who was evaluated for a three-month history of flaccid tetraplegia with a gradually ascending onset and for a deterioration in general health. electromyography findings were consistent with polyradiculoneuropathy. Laboratory tests showed a moderate amount of a monoclonal IgG-lambda antibody. Findings were normal from a radiographic bone survey and a radionuclide bone scan. Computed tomography of the pelvis disclosed a solitary osteolytic lesion in the right iliac crest, which was found upon biopsy to be a malignant plasmacytoma. radiation therapy and chemotherapy were given. Subacute or chronic polyradiculoneuropathy as the inaugural manifestation of solitary plasmacytoma is exceedingly rare and should be distinguished from the sensorimotor polyneuropathy produced by plasma cell infiltration in some multiple myeloma patients. The polyradiculoneuropathy of solitary plasmacytoma can be likened to the neuropathies seen in some forms of multiple myeloma (sclerotic myeloma and poems syndrome). The pathophysiology of these neuropathies remains obscure. The case reported here suggests that patients with unexplained lasting polyradiculoneuropathy should be investigated for a plasma cell proliferation even if they have no serum monoclonal component. Because plasmacytomas are painless, imaging studies are needed for their diagnosis. The management of the neuropathy consists in treatment of the tumor.
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ranking = 1.375
keywords = neuropathy
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15/1370. occupational exposure to methyl methacrylate monomer induces generalised neuropathy in a dental technician.

    A 36-year-old dental technician for 14 years developed paraesthesia and numbness in her legs. Neurophysiological studies revealed absent sensory nerve action potentials (SNAPs) from her lower limbs and normal upper limb SNAPs on presentation. Motor nerve studies were normal. Repeat studies 2 months after leaving her job showed some improvement in the lower limb SNAPs. It is suggested that her symptoms were caused by occupational exposure to methyl methacrylate monomer.
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keywords = neuropathy
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16/1370. Pulmonary manifestations of poems syndrome: case report and literature review.

    phrenic nerve paresis is an unusual complication of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein spike and skin changes) syndrome. In this report, we describe a case of poems syndrome in which a 56-year-old woman presented with dyspnea and ventilatory failure due to bilateral phrenic nerve paralysis. To our knowledge, only one other case of phrenic neuropathy in poems syndrome has been reported.
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ranking = 0.253019903147
keywords = neuropathy, gan
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17/1370. hypertrophy and pseudohypertrophy of the lower leg following chronic radiculopathy and neuropathy: imaging findings in two patients.

    Enlargement of the ipsilateral muscle compartment is an exceptional finding in patients with chronic radiculopathy, peripheral nerve injury, anterior horn cell diseases, or acquired peripheral neuropathy. We report radiographic, ultrasonographic, CT and MRI findings in a patient with chronic S1 radiculopathy and another with chronic neuropathy of the common fibular nerve (L4-S2), both presenting with painless enlargement of the calf muscles.
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ranking = 0.75
keywords = neuropathy
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18/1370. Clinical findings for a group of infants and young children with auditory neuropathy.

    OBJECTIVE: To examine the prevalence of auditory neuropathy in a group of infants at risk for hearing impairment and to present an overview of the clinical findings for affected children. DESIGN: Results for 20 subjects who showed repeatable cochlear microphonic potentials in the absence of click-evoked auditory brain stem responses are included in this study. Behavioral and steady state evoked potential thresholds were established in each case. Where possible, otoacoustic emission and speech perception results (unaided and aided) also were obtained. RESULTS: One in 433 (0.23%) of the children in our series had evidence of auditory neuropathy. The audiometric findings for these subjects varied significantly, with behavioral thresholds ranging from normal to profound levels. Discrimination skills were also variable. Approximately half of the subjects showed little understanding, or even awareness, of speech inputs in both the unaided and aided conditions. There were, however, a number of children who could score at significant levels on speech discrimination tasks and who benefited from the provision of amplification. CONCLUSION: The results suggest that auditory neuropathy is more common in the infant population than previously suspected. The effects of neuropathy on auditory function appear to be idiosyncratic, producing significant variations in both the detection and discrimination of auditory signals. As such, the management of children with this disorder must allow for individual differences.
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ranking = 1
keywords = neuropathy
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19/1370. Spinal root and plexus hypertrophy in chronic inflammatory demyelinating polyneuropathy.

    MRI was performed on the spinal roots, brachial and lumbar plexuses of 14 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). hypertrophy of cervical roots and brachial plexus was demonstrated in eight cases, six of whom also had hypertrophy of the lumbar plexus. Of 11 patients who received gadolinium, five of six cases with hypertrophy and one of five without hypertrophy demonstrated enhancement. All patients with hypertrophy had a relapsing-remitting course and a significantly longer disease duration. Gross onion-bulb formations were seen in a biopsy of nerve from the brachial plexus in one case with clinically evident nodular hypertrophy. We conclude that spinal root and plexus hypertrophy may be seen on MRI, particularly in cases of CIDP of long duration, and gadolinium enhancement may be present in active disease.
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ranking = 0.625
keywords = neuropathy
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20/1370. Chronic inflammatory demyelinating polyneuropathy with multiple hypertrophic nerves in intracranial, and intra- and extra-spinal segments.

    Hypertrophic nerves have occasionally been seen in chronic inflammatory demyelinating polyneuropathy (CIDP), but most are in the cauda equina. We report a case with CIDP in whom magnetic resonance imaging (MRI) with gadolinium diethylene triamine penta-acetic acid (Gd-DTPA) enhancement demonstrated hypertrophy of various peripheral nerves including multiple cranial nerves. Interestingly, none showed neurological signs corresponding to the lesions, except for clinical signs consistent with CIDP. MRI can be useful for the detection of silent, but abnormal nerve involvement in CIDP.
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ranking = 0.625
keywords = neuropathy
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