1/9. Familial amyloidotic polyneuropathy (ATTR Ser50Ile): the first autopsy case report.We report an autopsy case of a pedigree of familial amyloidotic polyneuropathy (FAP) with a mutation of isoleucine-50 transthyretin (ATTR Ser50Ile). A 47-year-old man started developing severe diarrhea and weight loss at age 41 years, followed by urinary incontinence, autonomic-nervous-system abnormalities and serious heart failure; the diagnosis of FAP (ATTR Ser50Ile) was made on the basis of genetic, histochemical and immunohistochemical analysis. Six years after the initial symptoms, he died of septic shock. autopsy revealed suppurative peritonitis, perforation of the sigmoid colon and marked systemic amyloid deposition. The total amount of amyloid deposited in the heart was greatly increased and was much lower in the thyroid gland and kidneys compared with amyloid deposits in ordinary FAP (ATTR Val30Met). amyloid deposition in peripheral vessel walls was prominent, particularly in lymphatics and veins. His elder sister, 54 years old, started to develop orthostatic hypotension at age 49 years, followed by dysesthesia, diarrhea and severe congestive heart failure. Endomyocardial biopsy revealed severe TTR-amyloid deposition; ultrastructural examination demonstrated that amyloid fibrils were deposited disproportionately and extended radially around microvessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Severe polyneuropathy in a patient with churg-strauss syndrome.We describe the clinicopathologic features of a 56-year-old woman affected with churg-strauss syndrome with major peripheral nerve involvement. The patient presented with a 1-month history of mainly distal upper-limb symmetrical paresthesias and hypostenia (bilateral "wrist drop"), palpable purpura and eosinophilia. Multiple pulmonary infiltrates and asthma had been present since the age of 52. skin biopsy demonstrated an eosinophilic necrotizing vasculitis. During the hospitalization she was submitted to cardiac, bronchopulmonary, renal, and gastrointestinal evaluation and EMG. Peripheral nerve and skeletal muscle biopsies were performed. sural nerve biopsy showed a marked degree of demyelination. A perivascular cellular infiltrate within the epineurium was immunoreactive for T lymphocytes and macrophages. Strong HLA-DR immunostaining was present in the endoneurium. IgM, IgE and fibrinogen deposition was found in some epi- and endoneurial vessels. Muscle biopsy showed neurogenic changes and 1 thrombosed vessel surrounded by mononuclear cells. Membrane attack complex (MAC) deposition was present in a few capillaries and major histocompatibility complex products I (MHCP I) was expressed at the subsarcolemmal level in a few isolated perivascular muscle fibers. After immunosuppressive therapy, the patient showed progressive improvement of both clinical symptoms and neurophysiological parameters.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/9. carbon disulfide vasculopathy: a small vessel disease.We present the clinical manifestations of 4 male patients with acute stroke-like symptoms and polyneuropathy after long-term exposure to carbon disulfide (CS2) in a viscose rayon plant. The ages of onset of polyneuropathy ranged from 42 to 45 years with a duration of CS2 exposure between 6 and 21 years. The ages of onset of stroke were from 42 to 48 years. The risk factors for stroke including heart disease and diabetes were denied, except for smoking in 4, hyperlipidemia in 2 and hypertension in 1. At the initial visit in 1992, only 2 patients developed sudden onset of hemiparesis suggesting a lacunar stroke before the diagnosis of CS2 intoxication. brain computed tomography (CT) scans showed low-density lesions in the basal ganglia in 2 patients, cortical atrophy in 1 and normal in 1. brain magnetic resonance image (MRI) study disclosed multiple lesions in the corona radiata and basal ganglia on T(2)-weighted images in 3 patients and cortical atrophy in 1. After the diagnosis, they left their jobs for a CS2-free environment, and improvement of the working conditions was noted. During 5 years follow-up period, another 2 patients also developed an acute episode of stroke with hemiparesis. brain CT and/or MRI follow-up studies in these 2 patients revealed new lesions in the basal ganglia and corona radiata. Intriguingly, a patient with previous stroke also developed new lesions in the bilateral thalami and brainstem. Carotid Doppler scan, transcranial Doppler scan and/or cerebral angiography did not show any prominent stenosis or occlusion in the major intracranial large arteries. We conclude that encephalopathy may occur in patients after long-term CS2 exposure, probably due to impaired cerebral perfusion. The lesions tend to occur in the basal ganglia, corona radiata and even brainstem, particularly involving the small-sized vessels. In addition, the cerebral lesions may progress even after cessation of CS2 exposure. Therefore, we suggest that CS2 exposure may be a risk factor for stroke.- - - - - - - - - - ranking = 2.5keywords = vessel (Clic here for more details about this article) |
4/9. sarcoidosis of the peripheral nerve: clinical, electrophysiological and histological study of two cases.Two cases of sarcoid polyneuropathy were diagnosed by histological examination of the nerve biopsy. The electrophysiological findings in both patients suggested a neuropathy of axonal type, confirmed by a morphological study of a sural nerve biopsy by light microscopy and on teased-fiber preparations. The sarcoid granulomas were multiple, especially in case 2; they were sparse in the epineurial and perineurial spaces and absent in the endoneurium, whose interstitial component contained cellular infiltrations of scattered macrophages. blood vessel changes were a constant morphological feature. The mechanisms that possibly contribute to the pathogenesis of the sarcoid neuropathy are discussed in relation to previous reports.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
5/9. Demyelinating polyneuropathy associated with monoclonal IgM-paraproteinaemia. Histological, ultrastructural and immunocytochemical studies.Histological, ultrastructural and immunocytochemical findings of sural nerve biopsies from 2 patients with monoclonal IgM-paraproteinaemia are presented. In both cases the pathological IgM antibodies reacted with a myelin antigen which was identified as myelin-associated glycoprotein (MAG) by immunoelectroblot . histology and electron microscopy showed typical features of a chronic demyelinating neuropathy with accompanying axonal degeneration. Immunohistochemical studies demonstrated IgM in the vicinity of endoneurial vessels and on some of the myelinated fibres. The localisation of IgM on the myelin sheath showed a typical pattern, which was similar to that found in binding studies with the patients sera on control nerves. It resembled the characteristic immunocytochemical staining pattern of MAG. Binding studies with the patients' sera on human and canine CNS material exhibited a clear labelling of white matter and certain, as yet unidentified structures within the cerebral and cerebellar cortex. In mixed glial cell cultures, the sera of both patients bound specifically to oligodendrocytes. Our observations are interpreted as immunohistochemical evidence that the anti-MAG antibodies of the patients' sera had bound to their antigenic target in the peripheral nerves. Because the antibodies clearly react to central myelin, oligodendrocytes and other not yet identified cortical structures, CNS involvement in such disorders should be considered.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
6/9. Sensorimotor polyneuropathy in light chain multiple myeloma.The authors describe an adult onset sensorimotor polyneuropathy in a woman with light chain K-type myeloma. Neurophysiological investigation showed a severe reduction of conduction velocity of the peripheral nerve to the four limbs. A muscle and sural nerve biopsy were performed. sural nerve displayed a severe loss of myelinated and unmyelinated fibers. No amyloid deposit was observed in vessels, perineurium and endomysium by electron microscopy examination. No onion bulb formation was present in nerve tissue, but many demyelinated fibers were surrounded by circumferentially disposed Schwann cell processes without any interposition of collagen fibrils. Some fibers exhibit signs of active remyelination; axonal alterations were rarely seen. collagen pockets were frequently observed among proliferated collagen fibrils. The mechanism of peripheral nerve lesions in various categories of multiple myeloma is till now an unsolved problem. In this case the unusual association with a light chain myeloma suggest that these immunoglobulins play a role in the pathogenesis of the polyneuropathy as supported by experimental studies.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
7/9. Cryoglobulinemic neuropathy: a pathological study.A 53-year-old woman developed symmetrical polyneuropathy of the lower limbs a few months after she was found to have myeloma with cryoglobulinemia. In musculocutaneous nerve biopsy material, electron microscopy showed both axonal degeneration and demyelination. The most striking finding was the presence, in the endoneurial space, of numerous masses of closely packed tubular structures. These masses also were found in the walls of all the vasa nervorum and within the lumen of some vessels. The morphological features and dimensions of the deposits within nerve were identical to those of cryoprecipitates extracted from serum and examined with the electron microscope. An example of myeloma neuropathy with cryoglobulin deposits within the endoneurial space has not been reported previously.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
8/9. Systemic lupus erythematosus with ischemic peripheral neuropathy and lupus anticoagulant: response to intravenous prostaglandin E1.We report a case of systemic lupus erythematosus with cutaneous vasculitis and peripheral neuropathy. Results of laboratory examinations revealed lupus anticoagulant in the patient's serum. His lower limbs showed asymmetry. A sural nerve biopsy specimen showed modest degeneration of nerve fibers and incomplete obstruction of nutrient vessels. His cutaneous vasculitis responded to treatment with high-dose corticosteroid, but the lower limb neuropathy responded incompletely. Daily intravenous prostaglandin E1 treatment improved his neurologic and cutaneous symptoms markedly. Our case implied that nutritional vascular ischemia caused by lupus anticoagulant is involved in the pathogenesis of lupus neuropathy in addition to well-known peripheral nerve vasculitis. It also documented the effectiveness of intravenous prostaglandin E1 in the treatment of systemic lupus erythematosus.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
9/9. Detection of borrelia burgdorferi dna and complement membrane attack complex deposits in the sural nerve of a patient with chronic polyneuropathy and tertiary lyme disease.We report a patient who developed a chronic sensory-motor polyneuropathy and a progressive myelopathy 4 years after a tick bite. An increased serum antibody titer to borrelia burgdorferi suggested a diagnosis of lyme neuroborreliosis, although a concomitant cervical spondylosis probably contributed to spinal cord damage. Treatment with ceftriaxone resulted in a marked improvement of neuropathic symptoms, providing indirect evidence of spirochetal infection. Search for B. burgdorferi dna by polymerase chain reaction amplification on sural nerve confirmed the diagnosis, demonstrating that the spirochete localized in the peripheral nervous system. The presence of complement membrane attack complex deposits and macrophage infiltrates around epineurial vessels and within the endoneurium suggests that the neuropathy in our patient was immune-mediated.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |