1/20. "Pseudo-conduction block" in vasculitic neuropathy.The predominant electrophysiologic feature of vasculitic mononeuropathy multiplex is axonal loss. Electrophysiologic findings interpreted as conduction block have, however, also been reported to occur in neuropathy secondary to necrotizing vasculitis. We report 3 patients with mononeuropathy multiplex and biopsy proven vasculitis in whom eight nerves met criteria for conduction block. In each circumstance, serial study demonstrated conversion of the electrophysiologic findings to those most consistent with severe axonal loss. "Conduction block" in vasculitic mononeuropathy multiplex is secondary to focal axonal conduction failure presumably related to infarctive axonal injury. The term conduction block should be used with caution in this disorder and only if serial studies demonstrate findings consistent with this electrophysiologic diagnosis.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/20. Endoneurial vasculitis and tubuloreticular inclusions in peripheral nerve biopsy.We describe 3 patients in whom nerve biopsy revealed endothelial tubuloreticular inclusions in association with peripheral nerve endoneurial vasculitis. Two of the patients had systemic lupus erythematosus (SLE), while the third was hiv-positive. review of our biopsy material featuring the much more common finding of epineurial vasculitis failed to disclose any instances in which endothelial tubuloreticular inclusions (TRIs) were present. We conclude that TRIs and endoneurial vasculitis are closely associated. Moreover, if detected on a nerve biopsy specimen, TRIs are very suggestive of SLE or hiv infection. Finally, literature evidence is cited to suggest that an "acid-labile" alpha-interferon may be pathogenically related to the vasculitic process in these patients, perhaps through a process mediated by tumor necrosis factor.- - - - - - - - - - ranking = 7keywords = nerve (Clic here for more details about this article) |
3/20. Acute axonal neuropathy in maple syrup urine disease.A 25-year-old woman with maple syrup urine disease (MSUD) developed generalized weakness over 1 week. She had severe leg and moderate arm weakness, areflexia, and distal sensory loss. plasma branched-chain amino acid concentrations were elevated, reflecting an acute exacerbation of the disease. Electrodiagnostic studies indicated an acute axonal polyneuropathy and sural nerve biopsy revealed acute wallerian degeneration without inflammation. Peripheral neuropathy, although not identified previously as a clinical feature of MSUD, may become more common as chronic dietary restrictions and improved management of the disease allow survival into adulthood.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/20. "Pseudo-conduction block" in a patient with vasculitic neuropathy.A 63-year-old man presented with progressive asymmetric weakness and numbness in his hands of 2 weeks duration. Nerve conduction studies showed low amplitude motor evoked potentials of both median nerves. The right ulnar, left tibial and peroneal nerves had normal potentials on distal stimulation with markedly decreased amplitudes proximally, suggestive of "conduction block". Three weeks later, amplitudes were decreased throughout. The patient was diagnosed with vasculitis. The acute ischemic injury presumably resulted in axonal damage between the distal and proximal stimulation sites, with subsequent wallerian degeneration.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
5/20. Chronic inflammatory polyradiculoneuropathy.The diagnostic criteria, natural history, nerve conduction characteristics, pathology, laboratory features, and efficacy of corticosteroid treatment have been evaluated personally in 53 patients with chronic inflammatory polyradiculoneuropathy (CIP) who were followed up for an average of about 7.5 years. These were patients whose monophasic neurologic deficit had not crested by 6 months, patients with recurrences, and patients with a steady or stepwise progression. The typical features of CIP include absence of an associated disease, frequent history of preceding infection or receipt of foreign protein, and tendency to involve cranial, truncal, and proximal as well as distal limb structures and to have diffusely slow conduction velocity of peripheral nerves. The most marked slowing is often very proximal. The pathologic features include serous edema, mononuclear cell infiltrates (especially in perivascular areas, but without evidence of vasculitis), macrophage-induced segmental demyelination, and hypertrophic neuritis. If our patients are representative, complete recovery occurs only infrequently; about 60% of patients are able to be ambulatory and work, 25% become confined to a wheelchair or become bedridden, and approximately 10% die from their disease. Although the bulk of the pathologic changes affect spinal roots and proximal nerves, the brain and spinal cord may be involved also. Degeneration into linear rows of myelin ovoids is the predominant type of myelinated fiber degeneration of the sural nerve at the ankle.- - - - - - - - - - ranking = 4keywords = nerve (Clic here for more details about this article) |
6/20. wallerian degeneration in human nerves: serial electrophysiological studies.After nerve transection, the distal stump undergoes wallerian degeneration (WD). Little information is available concerning sequential changes in nerve conduction measurements during WD in humans. Five patients with nerve injuries were studied temporally. Motor-evoked amplitudes were reduced by 50% at 3 to 5 days after injury; the response was absent by day 9. Sensory-evoked amplitudes were reduced by 50% at 7 days after injury; the response was absent by day 11. Sensory and motor nerves with shorter distal stumps showed earlier loss of amplitude than did those with longer distal stumps. denervation potentials were seen 10 to 14 days after injury. Our results suggest that WD occurs earlier if the distal stump is shorter, and that motor-evoked responses are affected earlier than sensory-evoked responses. The time-lag between the loss of the motor-evoked response and the appearance of denervation potentials, the latter coinciding with reduction of sensory evoked responses, suggests that failure of neuromuscular transmission precedes axonal loss during WD.- - - - - - - - - - ranking = 8keywords = nerve (Clic here for more details about this article) |
7/20. pathology of the sympathetic nervous system corresponding to the decreased cardiac uptake in 123I-metaiodobenzylguanidine (MIBG) scintigraphy in a patient with parkinson disease.Decreased cardiac uptake in (123)I-metaiodobenzylguanidine (MIBG) scintigraphy has been adopted as one of the most reliable diagnostic tests for parkinson disease (PD) in japan. To investigate the morphological basis for this finding, we performed a detailed neuropathological study of the cardiac sympathetic nervous system of a 71-year-old autopsy-proven PD patient, who presented with a marked decrease in cardiac uptake of MIBG, just 1 year prior to death. We carefully examined the intermediolateral column at several levels of the thoracic spinal cord, the sympathetic trunk and ganglia, and the nerve plexus of the anterior wall of the left ventricle and compared the findings with those of five age-matched controls. We found that the cardiac plexus was more heavily involved than the sympathetic ganglia in this patient with PD. Our study may provide further evidence that the markedly decreased cardiac uptake of MIBG observed in PD cases represents preferential involvement of the cardiac sympathetic nerve plexus in this disorder.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
8/20. The natural history and long-term outcome of 57 limb sarcoidosis neuropathy cases.Fifty-seven patients with biopsy-proven sarcoidosis causing limb neuropathy were reviewed in order to delineate the characteristic symptoms, impairments, disability, course, outcome and response to corticosteroid treatment of limb sarcoid neuropathy. Typically the neuropathy had a definite date of symptomatic onset. Prominent were positive neuropathic sensory symptoms (P-NSS), especially pain, overshadowing weakness and sensory loss. P-NSS were the main cause of disability. Almost always the pattern was asymmetric and not length-dependent (unlike distal polyneuropathy). We inferred (from kind and distribution of symptoms, signs and electrophysiologic and other test results) that the pathologic process was focal or multifocal, involving most classes of nerve fibers and variable levels of proximal to distal levels of roots and peripheral nerves. Additional features aiding in diagnosis were: systemic symptoms such as fatigue, malaise, arthralgia, fever and weight loss; involvement of multiple tissues (i.e. skin, lymph nodes and eye); the patterns of neuropathy; MRI features; and ultimately tissue diagnosis. Axonal degeneration predominated, although an acquired demyelinating process was observed in 3 patients. For most cases, the disease had a chronic, monophasic course. MRI studies done in later years of affected neural structures were helpful in identifying leptomeningeal thickening, hilar adenopathy; and enlargement and T2 enhancement of nerve roots, plexuses, and limb nerves. Corticosteroid treatment appeared to ameliorate symptoms more than impairments. Several variables were associated with neuropathic improvement: CSF pleocytosis, short duration between symptom onset and treatment, and a higher grade of disability at first evaluation-a possible rationale for future earlier diagnosis and treatment.- - - - - - - - - - ranking = 4keywords = nerve (Clic here for more details about this article) |
9/20. Peripheral neuropathy in monoclonal gammapathy with cryoglobulinemia and arteritis.We report a patient with IgM gammapathy, cryoglobulinemia, Raynaud's phenomena, purpura hyperglobulinemica of the legs and polyneuropathy. Endoneural vasculitis with infiltrations of eosinophilic and neutrophilic granulocytes and an extensive loss or wallerian degeneration of myelinated nerve fibers were seen on histopathologic examination of a sural nerve biopsy specimen. The microscopic picture differed somewhat from that observed previously in cryoglobulinemic vasculitis. Although vasculitis is most often believed to represent an immunologically mediated lesion, we propose an alternate explanation, namely, that the disease manifestations in the present case were secondary to cold-induced effects of the cryoglobulin on the microcirculation.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
10/20. adult pigment type (Peiffer) of sudanophilic leukodystrophy. Pathological and morphometrical studies on two autopsy cases of siblings.Two autopsy cases of siblings with the adult pigment (Peiffer) type of sudanophilic leukodystrophy (SLD), which demonstrated the full-blown stage (case 1) and early stage (case 2) of demyelination, were examined. Numerous brown pigments deposited in demyelinated cerebral areas were characterized histochemically and ultrastructurally as lipofuscin and ceroid. Under the electron microscope formation of blebs due to myelin splitting associated with deposition of multilamellar myeloid bodies within them was a prominent feature in the demyelinated cerebral areas of case 2 as compared with case 1. However, various features of myelin degradation such as thinning, partial or complete circumferential myelin loss, and deposition of electron-dense material on the interperiodic lines were found in both cases. Blebs occurred in all layers of myelin, and axons were compressed by these blebs or the hydropically swollen inner lips of oligodendroglias. Oligodendroglias were relatively well preserved in the demyelinated and nondemyelinated areas in case 2, although the cytoplasm was hydropic. Many spheroids were present in demyelinated areas and were irregularly distributed in both cases. The peripheral nerves in case 1 presented essentially the same changes as those in the brain, although those in case 2 were not affected. Morphometrically, the results showed that hypomyelination was not the mechanism for this pigment type of SLD. One possible cause may be an accelerated ageing of the metabolic process of myelin turnover.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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