Peripheral Sensorimotor Polyneuropathy Research Articles

An EMG case report of lead neuropathy 19 years after a shotgun injury

The clinical picture of lead neuropathy was classically described as a painless progressive motor neuropathy with axonal loss. The literature review fails to demonstrate a consensus on the site of axonal loss. This is an EMG report of a patient who developed a late lead neuropathy after a shotgun injury. A 69-year-old Filipino, healthy, male nondrinker sustained a shotgun injury to his left elbow. Nineteen years later he developed abdominal pain, followed by generalized weakness, distal greater than proximal in the extremities, and impaired pin-prick, proprioception, and two-point discrimination. He became nonambulatory and totally dependent in daily activities. He was lost to follow-up for 2 years until January 1993 when he presented with a blood lead level of 84 micrograms/dL. EMG examination revealed a sensorimotor peripheral polyneuropathy with severe axonal loss. This case demonstrates that axonal loss is the predominant feature in lead neuropathy and the location of pathology is in the peripheral nerves.

Treatment of diabetic polyneuropathy with tolrestat, an aldose reductase inhibitor

The efficacy and safety of tolrestat, an aldose reductase inhibitor, were evaluated in 21 diabetic patients (11 men, 10 women; mean age, 54.9 years) with symptomatic diabetic peripheral sensorimotor polyneuropathy in a 24-week, open-label clinical trial. The patients received single daily oral doses of tolrestat (200 mg). Eighteen patients had their dose increased to 400 mg daily at week 16 of the study and the remaining three patients had their dose increased similarly at week 20. Tolrestat-induced changes in neuropathy were detected by ranking symptom severity, testing nerve condution velocity, and assessing expiration-to-inspiration (E/I) ratios. The following significant mean changes from baseline were seen after 24 weeks of treatment with tolrestat: increase in the motor nerve conduction velocity of the median, ulnar, peroneal, and tibial nerves, and in the mean score for all of the four nerves; increased median sensory nerve conduction velocity; decreased sensory neuropathic pain symptoms; decreased paresthesia; improved cardiovascular autonomic nerve function and improved E/I ratio; and decreased muscle weakness. The overall response to tolrestat was rated as good or excellent in 95% of the patients by both the investigator and the patients. Adverse effects and clinical laboratory abnormalities were few and not clinically significant. Thus tolrestat (200 to 400 mg daily) appears to be safe and effective in improving the neuropathic symptoms in diabetic patients with mild-to-moderate peripheral sensorimotor polyneuropathy.

Polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo‐obstruction: POLIP syndrome

We describe 5 individuals (from three separate families) with a progressive neurological disorder characterized by sensorimotor peripheral polyneuropathy, cranial neuropathies (external ophthalmoplegia, deafness), and the syndrome of chronic intestinal pseudo-obstruction. Magnetic resonance imaging showed widespread abnormality of the cerebral and cerebellar white matter in the 2 patients studied. Autopsy examination in 3 revealed widespread endoneurial fibrosis and demyelination in the peripheral nervous system, possibly secondary to axonal atrophy, and poorly defined changes in cerebral white matter (leukoencephalopathy). The cranial nerves and spinal roots were less severely involved and the neurons in the brainstem and spinal cord were intact. The fatal gastrointestinal dysmotility was due to a severe visceral neuropathy. We suggest that these patients manifested a hereditary disorder with distinctive clinical, radiological, and neuropathological features, and propose the acronym POLIP to emphasize the distinctive tetrad of polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo-obstruction.

Chronic myelomonocytic leukemia with polyneuropathy and IgA-paraprotein.

A 65-year-old woman had chronic myelomonocytic leukemia with peripheral neuropathy and IgA paraprotein with kappa type light chain. Plasma cells with "flaming" cytoplasma were seen in the bone marrow specimens. The findings suggest that chronic myelomonocytic leukemia may involve B-lymphocytes in the proliferative process and that it may be a clonal disease. Moreover, this case was associated with peripheral sensori-motor polyneuropathy. The disorder was responsive to prednisolone, as oral administration of prednisolone improved not only the peripheral polyneuropathy, but also the hematological findings. Serum vitamin B12 and lysozyme in the serum and urine were decreased by the steroid therapy. The administration of prednisolone is effective not only for polyneuropathy but also chronic myelomonocytic leukemia.

A clinical neurophysiologic study of tropical spastic paraparesis.

During a field study in the Seychelles Islands, 19 patients with tropical spastic paraparesis (TSP) were evaluated electrophysiologically. Methods of assessment included motor and sensory nerve conduction studies, electromyography, and analysis of the somatosensory evoked potentials after stimulation of the tibial and median nerves. The results showed that the most prominent feature of the disease, a spastic paraparesis, is accompanied by subclinical involvement of spinal sensory pathways and a comparatively minor peripheral sensorimotor polyneuropathy.

Pathogenic and clinical aspects of polyneuropathies, with reference to the hand-arm vibration syndrome.

Along with attacks of white finger, symptoms suggesting peripheral sensorimotor neuropathy, ie, polyneuropathy or entrapment neuropathy, are very important in the hand-arm vibration syndrome. Peripheral neuropathies are probably associated with the occurrence of the syndrome because of a selection mechanism. Polyneuropathy may be a contributing factor in the development of entrapment neuropathies in the upper extremities. It has multiple pathogenic mechanisms and numerous causative factors. However, peripheral nerves can react to pathological stimuli in a limited number of ways. Wallerian degeneration, segmental demyelination, and axonal degeneration are the classical neuropathological types of peripheral neuropathies, of which the first two are possible direct consequences of vibration exposure. The clinical manifestations of polyneuropathy range from sensory to motor types, sometimes with autonomic involvement. Whenever polyneuropathy is encountered in the hand-arm vibration syndrome, its etiologic possibilities should be considered. Regardless of the variable criteria used by different authors, individual diagnosis of the syndrome is always a probability diagnosis, and adequate neurological differential diagnostics have to be employed.

Relationship of tissue deposits of cryoglobulin to clinical features of mixed cryoglobulinemia

Two patients with type 2 mixed cryoglobulinemia had tissue deposits of serum cryoproteins. Patient 1, a 72-year-old man, had purpura and glomerulonephritis. The serum cryoglobulin consisted of monoclonal IgM kappa and polyclonal IgG. Renal biopsy revealed diffuse proliferative glomerulonephritis with abundant IgG, IgM, kappa light chain, and complement in glomerular capillary walls. These immunoglobulins, but no complement, were also present in histologically normal cutaneous blood vessels. Ultrastructurally, cutaneous vascular deposits were identical to the renal deposits and to the crystalline mixed IgM-IgG serum cryoprecipitates and renal deposits previously described. Patient 2 was a 68-year-old man with sensorimotor peripheral polyneuropathy and purpura. His serum cryoglobulin consisted of monoclonal IgA lambda and polyclonal IgG. Sural nerve and skin biopsies revealed vasculitis involving small arteries and arterioles. Immunoglobulin A and complement were present in perineurial arteriolar walls of the sural nerve. Cryoprecipitates in both cases had strong rheumatoid factor activity. These findings support the view that in type 2 cryoglobulinemia tissue deposits consist of cryoprotein immune complexes. The presence of these deposits in histologically normal blood vessels in patient 1 suggests that deposition of cryoproteins precedes and may initiate tissue damage.