1/49. Vasculitis confined to the peripheral nerve and skin: a variant of non-systemic vasculitic neuropathy.We describe a patient presenting with vasculitic neuropathy associated with cutaneous vasculitis in the absence of other clinical or laboratory evidence of the underlying systemic vasculitis, and showing a favourable prognosis. Although the early proposed criteria for the diagnosis of non-systemic vasculitic neuropathy (NSVN) exclude the involvement of extraneural tissues, the condition observed in our patient might represent a variant of NSVN, with the vasculitis confined to the small vessels in both the peripheral nerve and the skin.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/49. 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.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/49. Angiotropic large B-cell lymphoma with clinical features resembling subacute combined degeneration of the cord.Angiotropic large cell lymphoma is a rare neoplastic disorder associated with a high mortality. The hallmark of the disease is lymphoid proliferation confined to the intravascular compartment without local tissue or vessel wall infiltration [1]. This feature is so striking that the disease was originally thought to arise from endothelial tissue and early cases were described as malignant angioendotheliomatosis. However, application of immunohistochemical methods for detection of lymphoid markers such as the CD45 and CD20 cell surface markers has confirmed its lymphoid origin, usually of B-cell lineage [2]. Clinical manifestations of the disease are protean and are due to multifocal medium and small vessel occlusion by tumour cells [3]. Characteristic sites of involvement are skin and central nervous system and although an ante-mortem diagnosis can be made from a biopsy specimen, it is often unsuspected [4]. We present a case of angiotropic large B-cell lymphoma in a 74-year-old man who presented with urinary symptoms and had a neurological picture resembling subacute combined degeneration of the cord.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/49. Vasculitic neuropathy in a patient with inactive treated lepromatous leprosy.A 46 year old Asian male with previously treated lepromatous leprosy developed a stepwise multifocal sensory disturbance 25 years later. neurophysiology demonstrated marked deterioration from previous studies. sural nerve biopsy disclosed a vasculitic process superimposed on inactive lepromatous leprosy. Immunocytochemical stains for mycobacterial antigen showed deposits within nerve and vessel walls. A delayed vasculitic neuropathy precipitated by persisting mycobacterial antigen is proposed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/49. hepatitis c virus infection with peripheral neuropathy is not always associated with cryoglobulinaemia.OBJECTIVES: To describe cases of peripheral neuropathy associated with chronic hepatitis c virus infection without mixed cryoglobulinaemia. methods: Four cases of peripheral neuropathy associated with chronic hepatitis c virus infection with persistent negativity of mixed cryoglobulinaemia were found. RESULTS: All patients had small increases of transaminase levels and a positive viraemia. liver biopsy showed chronic active hepatitis in all but one case (Knodell 4-9, Metavir A0F0-A3F3). Neuromuscular biopsy showed axonal neuropathy associated with lymphoid infiltrates around small vessels in two cases. rheumatoid factor was always negative and C4 complement level was always normal. In three patients, neuropathy improved with interferon alpha, interferon alpha ursodesoxycholic acid, or steroids plasma exchange. CONCLUSION: Peripheral neuropathy may be associated with hepatitis c virus infection without mixed cryoglobulinaemia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/49. Fascicular torsion in the median nerve within the distal third of the upper arm: three cases of nontraumatic anterior interosseous nerve palsy.Three patients with nontraumatic anterior interosseous nerve palsy are presented. All patients also had paralysis of the pronator teres, flexor carpi radialis, and/or palmaris longus. One patient also had sensory disturbance and palsy of the thenar muscles. An hourglass-like constriction was seen within a 7-cm section of the nerve fascicles (2-9 cm proximal from the medial epicondyle of the humerus) in the median nerve trunk. All constrictions exhibited approximately 30 degrees of fascicular torsion. Because this nerve section is anatomically proximal to the branching point for the earlier mentioned motor branches and the anterior interosseous nerve, the nerve fascicles may have been structurally twisted before the onset of palsy. Structural abnormalities causing inflammation and edema of nerve fascicles as well as factors such as compression from surrounding small vessels may have maximized torsion, resulting in the formation of constrictions.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/49. cholesterol emboli neuropathy.We report the clinical and pathologic features of a patient with peripheral neuropathy that was the first clinical expression of cholesterol emboli syndrome (CES). biopsy of skeletal muscle and peripheral nerve revealed cholesterol clefts in lumens of small arteries, necrotizing arteritis, and severe degeneration of peripheral and intramuscular nerves. At autopsy, the peripheral nervous system was extensively affected by similar changes. We conclude that (1) peripheral neuropathy may be the initial manifestation of CES. Presumably, deposition of cholesterol leads to arteritis. (2) The underlying pathology of CES neuropathy is chronic axonal degeneration, possibly due to chronic ischemia of epineurial arteries. (3) Muscle biopsy is important in the antemortem diagnosis of CES. Nerve biopsy may show involvement of epineurial vessels. (4) CES may resemble polyarteritis nodosa clinically and pathologically. (5) CES may be under-recognized and should be included in the differential diagnosis of any neuropathy of uncertain cause, particularly when there is a history of vascular catheterization, or severe aortic atherosclerosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/49. Electrodiagnostic characteristics of Wegener's granulomatosis-associated peripheral neuropathy.Wegener's granulomatosis is a multisystem disease distinguished by a triad of necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts, glomerulonephritis and systemic, small vessel vasculitis. The latter can cause neurologic manifestations when the vasa nervorum are affected. A 53-yr-old male presented with a 3-mo history of chronic nasal congestion, arthralgias, pruritic maculopapular eruption, epistaxis and lower extremity weakness. Subsequent lung and chest wall biopsies confirmed diagnosis of Wegener's granulomatosis. Summary of electrodiagnostic data obtained on initial presentation and comparison with later study indicated a sensorimotor polyneuropathy with wide-spread axonal involvement noted particularly in the distal lower extremity musculature. Electrodiagnostic documentation of rapid progression proved useful in directing alteration of immunosuppressive therapy, with favorable clinical and functional outcome. We believe this is the second case presented of a patient with documented Wegener's granulomatosis and overt clinical evidence of poly-neuropathy in whom both electroneurographic and electromyographic studies are described. Electrodiagnostic results are presented with discussion of pertinent literature.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/49. Reversible conduction block in human ischemic neuropathy after ergotamine abuse.Conduction block [a significant reduction in compound muscle action potential (CMAP) amplitude after proximal compared to distal stimulation] is often found in demyelinating neuropathies, including inflammatory neuropathies and degenerative neuropathies, such as "liability to pressure neuropathy." There is experimental evidence that a transient conduction block can occur in rats after ischemic lesions of peripheral nerves are induced either by ligation of arterial vessels supplying nerve trunks, or by injection of arachidonic acid into peripheral arterial vessels. Conduction block has also recently been described in cases with necrotizing vasculitis. To date, however, no example of a reversible conduction block has been reported in human ischemic neuropathy.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
10/49. Neurogenic bladder due to peripheral neuropathy and a visual disturbance in an elderly man with systemic lupus erythematosus.A 63 year old man with central nervous system lupus with a neurogenic bladder and visual disturbance is described. The diagnosis of neurogenic bladder, attributed to peripheral neuropathy, was made on the basis of cystometrography and clinical symptoms. A brain magnetic resonance imaging scan showed gliosis along the cerebral vessels and the optic nerve. This case shows that systemic lupus erythematosus can be accompanied by a peripheral neurogenic bladder and visual disturbance, and that these symptoms may not improve despite the amelioration of other lupus symptoms on treatment with steroids.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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