[Guillain-Barré syndrome: chronic inflammatory demyelinating polyradiculoneuropathy].
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Biomedical subjects
Publications and source records attributed to K Kumazawa.
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IOH occurs as progressive autonomic failure (PAF) without any neurologic symptoms indicating multiple system atrophy or Parkinson's disease. The responsible lesion for IOH is yet obscure but has been suggested to be in the peripheral sympathetic nerves, since postganglionic sympathetic neurons in IOH fail to release norepinephrine and there present extensive supersensitivities to exogenous pressors. SOH is characterized as marked tachycardia induced by hypotensive stress like standing, and is less sensitive to the administered catecholamines. Careful examinations by some pharmacological studies are essential to diagnose IOH and SOH in patients with orthostatic hypotension.
We have reported two cases of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) associated with Graves' disease. Case 1: a 45-year-old woman noticed a diffuse goiter, palpitation and emaciation in 1977. Laboratory studies confirmed that she had Graves' disease, and she was treated with antithyroid drug. In 1986, when the hyperthyroidism was subsided, she showed progressive symmetrical weakness and numbness in her limbs, and she was almost in tetraplegia at 1987. Markedly slowed motor and sensory nerve conductions and elevated CSF proteins as well as clinical manifestations confirmed the diagnosis of CIDP. Following corticosteroid-pulse therapy and plasmapheresis resulted in good recovery in both motor and sensory impairment, though two-times of relapses were observed. Case 2: a 33-year-old man first noticed weakness in his legs in 1977, motor and sensory disturbances progressed for 12 years. Slowed nerve conduction, high CSF proteins and two-times of relapses in early phase indicated that the CIDP was the diagnosis. In 1989 he complained general fatigue, hyperhidrosis and body-weight loss. The serum thyroid hormone levels were high, and other laboratory studies confirmed the presence of Graves' disease. The cases with both CIDP and Graves' disease has rarely been reported. The background mechanism of this association is not well understood, but the susceptibility to CIDP and Graves' disease may be related to the HLA antigens and immunoglobulin Gm allotypes of which are the genes linked to the major histocompatibility complex and controlling immune responses. The present two cases commonly shared several HLA-DR antigens, but their significance should be confirmed by examining many cases.
The effect of local administration of calcitonin gene-related peptide (CGRP) on sweating activity was evaluated on normal human volunteers. CGRP and methacholine chloride (MCH) was dissolved in 0.1 ml of 0.9% NaCl solution to a specified concentration, and was injected intradermally at the center of a 1.3 cm2 forearm test area. The sweat rate was recorded continuously by capacitance hygrometry in a relatively cool environment (Ta, 23 degrees C). CGRP did not elicit any sweat secretion when administrated by alone, but significantly increased the sweat rate when it was administrated with MCH. The maximum enhancement of MCH-induced sweating by CGRP was observed at a concentration of 10(-5) g/ml of CGRP. There was clear dose-dependent relationship between the dose of CGRP and its enhancement. Recently, CGRP-like immunoreactivity is demonstrated to be present in cholinergic nerve terminals around the human sweat glands. These observations have strongly suggested that CGRP enhances the cholinergic sweating activity. Although the underlying mechanism is still obscure, CGRP may enhance the sweating as a consequence of vasodilation which has been known to be a major activity of CGRP. As for the evaluation of human sweat gland function, CGRP-induced peptidergic regulation should be considered as well as cholinergic regulation.
We examined autonomic functions in 14 patients with peripheral neuropathy caused by necrotizing vasculitis. These patients consisted of three allergic granulomatous angitis (Churg-Strauss syndrome, AGA), two systemic lupus erythematosus (SLE), two progressive systemic sclerosis (PSS), one mixed connective tissue disease (MCTD), one polyarteritis nodosa (PN) and five nonsystemic vasculitis. All of them were proven to have a vasculitis by sural nerve, muscle or skin biopsy. Sixteen age- and sex-matched healthy volunteers were also examined. Local sweating induced by intradermal injection of pilocarpine and nicotine (a concentration of 10(-4) g/dl, 0.1 ml) was measured with ventilated capsular method on the forearm and lower lateral leg at 23 degrees C of room temperature and 40% of relative humidity. Other autonomic functions including skin temperature at rest and after cold loading (15 degrees C, 6 minutes), variation in the R-R interval of heart beat (CV%), orthostatic hypotension and bladder dysfunction were also monitored. A decrease in sweat rate and recovery rate of skin temperature after cold-loading was seen more frequently in patients with necrotizing vasculitis than normal volunteers. Abnormality in the local sweat response against nicotine and pilocarpine was more frequently present in the involved area of somato-sensory and motor nerves as compared with those in the non-involved area. An occurrence of decrease in recovery rate of skin temperature after cold-loading also well correlated to the region of somato-sensory- and motor involvement. So far other autonomic dysfunction, only one patient had orthostatic hypotension, impotence and bladder dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)
The disappearance rate of indocyanine green (K.ICG) and the maximum removal rate (Rmax) usually correlate with each other. However, in some cases it was shown there was a dissociation between them. We investigated the relationship between the two rates in 146 subjects. K.ICG and Rmax correlated strongly with a correlation coefficient of 0.749 (p less than 0.001). Sixty-six cases were included in the limits of 95% confidence, and the other 80 cases outside the limits were defined as dissociated cases. Among them a lower Rmax rate as compared to the K.ICG rate was found in many cases of obstructive jaundice. Particularly a lower K.ICG rate compared to the Rmax rate was found in many cases of liver cirrhosis accompanied by esophageal varices and idiopathic portal hypertension. On the other hands, we performed multiple regression analysis on 12 other liver function tests. K.ICG was strongly related to platelet count, circulatory blood volume, and albumin, all factors relating to portal hypertension. Rmax largely depended on LCAT, A/G ratio, and cholinesterase, which are Therefore, the dissociation between K.ICG and Rmax was caused by differences in the characteristic of each disease.
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Multiple mononeuritis of migrating nature at onset occurs in a variety of disease conditions. "Migrant sensory neuritis" without a systemic underlying disorder described by Wartenberg (1958) is the most distinctive form of this type of neuropathy. Its pathomechanism is not uniform, but angiopathy has been suggested by Matthews et al. (1981). In this report, we describe five cases of sensory-dominant multiple mononeuritis with migrating nature and without systemic visceral involvement. The patients consisted of four females and one male between 26 and 71 years of age. All showed recurrent episodes of sensory involvement along the distal branches of the cutaneous nerve. The patients presented with sudden onset of numbness or pain radiating along the cutaneous branches of the involved nerves often followed by persistent sensory deficit. In mild episodes, the sensory symptom with numbness almost completely subsided. The frequency of these episodes ranged from 7 times in 6 months to 6 times in 9 years. The clinical manifestations were similar or identical to those of migrant sensory neuritis reported by Watenberg and Matthews et al. Cranial nerve involvement and less frequent episodes in the present series differed from those in the previous reports. Laboratory examinations did not disclose any underlying disorder, suggesting systemic collagen vascular disease. Sural nerve biopsy study in two patients revealed vasculitis in small or medium-sized arteries in the epineurium as well as reduced population of large myelinated fibers. Small myelinated fibers were slightly increased in number, probably to regenerating fibers. Sensory action potentials were low or not evoked in the nerves examined.(ABSTRACT TRUNCATED AT 250 WORDS)
We examined autonomic dysfunctions in 12 patients with chronic-recurrent-progressive polyradiculoneuritis (C-R-P-PRN), consisting of 6 males and 6 females with the clinical duration of 8 months to 16 years, and with the age ranged 13 and 71 years. Sixteen healthy volunteers, aged 20 to 70 years, were also examined. Thermal sweat rate was recorded on the palm, forearm, upper arm, anterior chest, lateral thigh and lateral aspect of lower leg using a ventilated capsular method in a climatic chamber at 40 degrees C and 40% of relative humidity. After steady state was attained, thermal sweat rate was measured. Local sweating induced by intradermal injection of pilocarpine and nicotine (a concentration of 10(-4), 0.1 ml) was also measured on the forearm and lower lateral leg at 23 degrees C of room temperature and 40% of relative humidity. Other autonomic functions including skin temperature at rest and after cold loading (15 degrees C, 6 minutes), variation in the R-R interval (CV%), pupillary function (response to 1.25% epinephrine, 2(-5) pilocarpine, 5% tyramine), orthostatic hypotension and bladder dysfunction were also monitored. A decrease in sweat rate and recovery rate of skin temperature was seen more frequently in patients with C-R-P-PRN than normal volunteers. Abnormality in the thermal sweat rate and local sweat response against nicotine and pilocarpine was present more frequently in the forearm and distal leg as compared with the chest and thigh.(ABSTRACT TRUNCATED AT 250 WORDS)
Postganglionic sudomotor function were examined on 12 patients with multiple system atrophy (MSA) consisting of 5 males and 7 females with the clinical duration of 1 to 10 years, and with the age ranged 51 and 70 years. Sixteen healthy volunteers, aged 38 to 75 years were also examined as the control. Local sweating induced by intradermal injection of pilocarpine and nicotine (a concentration of 10(-4) g/ml) was quantitatively measured on the volar surface of the forearm and lower lateral leg using a ventilated capsule method in a climatic chamber at 23 degrees C and 40% of relative humidity. Maximal sweat rate induced by nicotine and pilocarpine was significantly reduced in patients with MSA as compared with controls in both the forearm and lower lateral leg. MSA cases associated with more prominent autonomic dysfunction as well as hyposweating, showed a more remarkable impairment of local sweat responses. Particularly, in 6 cases with Shy-Drager syndrome, there was no sweat response by the injection of both pilocarpine and nicotine. The study of an autopsied case with Shy-Drager syndrome revealed neurons in the para-vertebral sympathetic ganglia were well populated, though neurons in the lateral horns of the lower thoracic spinal cord were almost completely depleted. This substantial discrepancy between the impaired sudomotor function and morphological findings may imply several hypothetical views on the mode of pathology of postganglionic sudomotor nerves. The present results, however, strongly suggested that postganglionic sudomotor functions are more extensively involved in patients with MSA than had ever been believed.(ABSTRACT TRUNCATED AT 250 WORDS)
Time-associated changes in the disappearance rate of indocyanine green from the blood (K.ICG) as an index of liver function, were studied. Blood was drawn 5 times at 3-minute intervals from 32 patients. Early, intermediate, and late K.ICG values were 0.087 +/- 0.040, 0.082 +/- 0.038, and 0.076 +/- 0.033 min-1, respectively, showing serial decreases. When blood was drawn 8 times at 2-minute intervals from 22 other patients, the means of the K.ICG values at 11 time points showed a nearly linear relationship (r = -0.986). These findings indicated that K.ICG is approximated by a linear function of time, K(t) = -K'.t + K0. According to this function, K.ICG is considered to decrease by 1.96% every minute. The K.ICG value determined by the conventional method is, therefore, a mean disappearance rate of 15 minutes, and K0 is considered to reflect the initial reaction speed.
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