1/8. Amyloid myopathy presenting with distal atrophic weakness.Amyloid myopathy is a rare complication of primary amyloidosis usually presenting with proximal muscle weakness. We report a woman with multiple myeloma in whom marked atrophy and weakness of finger flexor muscles were the first manifestations of systemic amyloidosis. Muscle biopsy revealed amyloid angiopathy with deposits of lambda light chains in vessel walls. The recognition of amyloid myopathy is important because clinical symptoms may respond to chemotherapy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/8. A case of progressive muscular dystrophy with numerous arterioluminal vessels.A 48-year-old man had histologically demonstrated cardiac involvement associated with progressive muscular dystrophy. On coronary arteriography, numerous vascular communications between the coronary arteries and the left ventricular chamber were found. These vascular communications are considered to be the arterioluminal vessels. This is the first report of a case of progressive muscular dystrophy with numerous arterioluminal vessels.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
3/8. erythema gyratum perstans: association with a familial neurologic disease.Two members of the same family with erythema gyratum perstans and hypertrophic neuritis are reported. The dermatosis could be an expression of localization of neuritis to nerva vasorum with abnormal neurovascular response of cutaneous small vessels to normal stimuli with active erythema followed by cyanosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/8. "Epi-" and intramedullary neurilemmoma of the spinal cord with denervation atrophy in the related skeletal muscles.The autopsy of a 68-year-old male who died of cardiac infarction revealed an ep - and intramedullary neurilemmoma of the spinal cord as an associated finding half a year prior to death. The patient had suffered from progressive weakness and sensory disturbances of the lower limbs together with muscular wasting for 6 months. Repeated neurological examinations had led to the diagnosis of an intraspinal space occupation which, however, could not be substantiated by myelography because of its surprisingly small size. The Schwann cell proliferation originated from the adventitia of the epi- and intramedullary vessels of the conus medullaris. The main tumor mass was epi-medullary and extended into the medullary parenchyma via the penetrating vessels forming intramedullary nodules. The special findings in the present case seem to support the hypothesis that intramedullary neurilemmomas originate from the perivascular nerve endings.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
5/8. Rigid spine syndrome: histological examinations of male and female cases.Histological findings in the limb-muscle of two new cases of Rigid spine syndrome are described. The first male case exhibited a disproportion in fiber type with predominance of the type II fibers with a slight increase of large fibers. Some of the type I fibers were slightly atrophic. No other consistent abnormalities could be seen, excepted thickness of vessel basal lamina. The second female case had a relatively distinct muscular pattern. Necrotizing phenomenon were mild without fibrosis. Type I predominance were obvious. Atrophic and hypertrophic fibers could be seen in both types populations, with a relatively higher rate in type II. These two additional cases and those of the literature showed varied non specific histology, without any histo-chemical nor ultrastructural characteristic pattern.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/8. Functional alterations of muscle perfusion in type II muscle atrophy: demonstration by lower extremity perfusion scans.Significant progress in the clinical assessment of peripheral vascular disease has resulted from the use of radionuclide scans to evaluate alterations of blood flow through small vessels. Two patients with Type II muscle atrophy and normal vessels on muscle biopsy were examined; abnormal lower extremity perfusion scans were obtained after the administration of 99mTc-labeled human albumin microspheres directly into the femoral arteries. These case studies illustrate the use of this technique for delineating muscle groups affected by a myopathic process. Muscle perfusion scans can be used as an aid in selecting biopsy sites and may provide a means of following the response to therapy.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/8. Facioscapulohumeral syndrome with sensorineural hearing loss and abnormality of retinal vessels.A 23-year-old man was recognized as a sporadic case of facioscapulohumeral syndrome (FSH syndrome). He was a case of early onset, and there was severe atrophy of skeletal muscles, although muscle biopsy was normal. Electromyogram revealed neurogenic changes. This patient also had severe sensorineural hearing loss and a mild abnormality of retinal vessels. It is known that some FSH dystrophy or FSH spinal muscular atrophy patients have such coincidental complications. The cause of these coincidences is still unclear, but their occurrence suggests the pleiotropy of the FSH syndrome.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
8/8. Peripheral neuropathy in scleroderma.nervous system involvement is rare in progressive systemic sclerosis (PSS). We present a clinical pathological and immunological study of two patients with peripheral sensory motor neuropathy and PSS. In both, the sural nerve biopsies showed axonal degeneration with increased endoneurial connective tissue. There were also clusters of myelinated fibres indicating axonal regeneration. Only mild microangiopathic changes were evident in the endo, peri and epineurial vessels. By Western immunoblots, patients' sera contained a band of reactivity to a protein from peripheral nerve identified as collagen type i. Primary involvement of the peripheral nerves during PSS is very unusual. Abnormal production of collagen tissue and presence of microvascular disease are considered to be two possible causes of neuropathy. We think that our results suggest the important role of the connective tissue proliferation in the pathogenesis of PSS neuropathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |