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T Kohriyama

Publications and source records attributed to T Kohriyama.

At least 37 records · Page 2Linked to original sources
PubMed1997

[A case of optic-spinal form of multiple sclerosis with lobar type large cerebral hemorrhage].

We report a 57-year-old woman with optic-spinal form of active multiple sclerosis, who developed a large lobar type hemorrhage of the brain. She initially suffered from left visual loss, and three month later, she was hospitalized with paraplegia and total sensory loss up to the fourth thoracic level accompanied by sphincteric disturbance. Diagnosis of clinically probable multiple sclerosis was based on the relapsing-remitting clinical course and laboratory findings. Five months after admission, she developed sudden consciousness loss. Brain CT scan showed a massive hemorrhage in the right frontal to parietal lobe. The patient had no risk factors for cerebral hemorrhage including hypertension. Histopathological study of brain tissues obtained at surgical evacuation of hematoma did not reveal any malignancy, and congo-red staining of this specimen was negative. We analyzed coagulation, fibrinolytic, and endothelial parameters during the follow-up period. von Willebrand factor (vWF) as a marker for endothelial damages was elevated persistently. Moreover, thrombin-antithrombin III complex (TAT) as a marker for activation of coagulation was also elevated constantly throughout the clinical course. The findings suggest that fragility of the vascular walls and permeability changes associated with immunological mechanisms might have resulted in the cerebral hemorrhage. Although there are few reports of cerebral hemorrhage in patients with multiple sclerosis, it has been reported that vascular wall damage is an important aspect of the pathology of multiple sclerosis and acute cerebral vascular damage may sometimes occur in multiple sclerosis. We propose that coagulation studies including the endothelial marker such as vWF would provide a useful information regarding the risk of cerebrovascular complication in multiple sclerosis.

Blood Coagulation Tests↗
PubMed1996

[Coagulation and fibrinolytic parameters as predictors for small-vessel disease revealed by magnetic resonance imaging of the brain].

We correlated coagulation and fibrinolytic parameters with small-vessel disease revealed by magnetic resonance imaging (MRI) of the brain. One hundred and eleven patients with asymptomatic or symptomatic cerebral infarction were randomly selected for the study; 57 males and 54 females with an average age of 66.6 +/- 9.6, age range 40 to 85, years old. Among them, 76 patients had a history of symptomatic cerebral infarction; 38 patients hypertension; and 24 patients diabetes mellitus. Patients with large cortical infarction, cerebral hemorrhage, demyelinating disease or mass lesions were excluded from the present study. The MRI scans were reviewed for areas with increased signal intensity on T2-weighted images. The small infarction was defined as a lesion less than 10mm in diameter. The activity of von Willebrand factor (vWF) correlated significantly with the grade of caps at the anterior and posterior horns of the lateral ventricle, and the number of small infarctions in the subcortical white matter and basal ganglia, suggesting vWF could be a predictor for these small-vessel disease. The grade of caps at posterior horn of the lateral ventricle and the number of small infarctions in the subcortical white matter were associated significantly with the concentration of plasma fibrinogen and reversely with the activity of antithrombin III, an inhibitory factor in coagulation system. These results indicate that hypercoagulable state may causatively relate with small-vessel disease in the territory of medullary artery branching from cortical artery. On the contrary, these coagulation parameters did not correlate significantly with small ischemic lesions in the territory of perforating artery. No correlation was observed between the level of marker proteins for platelet activation and the degree of small-vessel disease, indicating the activation of platelet could not associate with the etiology of small-vessel disease.

Adult↗
PubMed1996

[Significance of the coagulation and fibrinolytic parameters as predictors for carotid atherosclerosis].

We investigated the relation between atherosclerosis of common carotid artery measured by B-mode ultrasound sonography and vascular risk factors, in particular the coagulation and fibrinolytic parameters. A total of 118 patients without either hematological disease or symptomatic brain infarction are enrolled in this study. Vascular risk factors associated with increased intima-media thickness (IMT) of common carotid artery were advancing age, sex (male), systolic blood pressure and diastolic blood pressure. There was a significant correlation of IMT with the activity of von Willebrand factor (vW factor) which had a significant correlation with concentration of beta-thromboglobin (beta TG) and platelet factor 4 (PF4). It has been reported that vW factor is a useful parameter for the evaluation of the extent of vascular involvement. vW factor in plasma and in the vessel wall is known to contribute appreciably to the platelet adherence, and to play a major role in the platelet aggregation particularly at high shear stress. In this study, we showed that plasma vW factor might enhance platelet aggregation. In addition, we revealed that plasma vW factor might reflect the injury of carotid artery wall and serve as a significant predictor of carotid atherosclerosis. There was a significant correlation of IMT with concentration of fibrinogen (Fbg), suggesting Fbg may play a role in atherogenesis by affecting local blood flow especially at the bifurcation. We reconfirmed that Fbg is a significant indicator of carotid atherosclerosis. Concentrations of beta TG and PF4 were significantly correlated with IMT. They are useful parameters of platelet activation and elevated in various angiopathies. These results, therefore, indicate that beta TG and PF4 also reflect the injury of carotid artery wall, and could be markers for carotid atherosclerosis.

Arteriosclerosis↗
PubMed1996

[Autosomal recessive early-onset parkinsonism with diurnal fluctuation (AR-EPDF)--clinical characteristics].

Among heterogeneous diseases manifested by parkinsonism beginning early in life, there is a disease presenting with marked diurnal fluctuation of symptoms, called autosomal recessive early-onset parkinsonism with diurnal fluctuation (AR-EPDF). To identify the characteristics of this condition as a disease entity, we examined the clinical manifestations of AR-EPDF patients (Group I, n = 42) in comparison with those of early-onset parkinsonism patients without diurnal fluctuation (Group II, n = 34). Family history suggesting autosomal recessive inheritance was noted in 85.7% of Group I patients and 17.6% of Group II. The male-to-female ratio was 1: 1.8 in Group I, and 1:0.89 in Group II. Age at onset showed a standard distribution with an average of 25.6 years (SD: +/- 7.7) in Group I and an average of 32.7 with an increasing pattern toward 40 years in Group II. The initial symptom was dystonic gait disturbance in 42.9% of Group I and 5.9% of Group II, parkinsonian gait in 19.9% of Group I and 2.9% of Group II, and tremor in 28.6% of Group I and 41.2% of Group II. The main clinical feature was parkinsonism in both groups. Diurnal fluctuation of parkinsonism was remarkable in all but one (97.6%) of Group I, while it was not observed in Group II. Dystonic postures were noted in 79.4% of Group I and in 37.1% of Group II; hyperactive tendon reflexes in 74.3% of Group I patients and in 20% of Group II. Autonomic symptoms were mild in both groups. None of the Group I patients had dementia while two of Group II did. Levodopa was markedly effective in both groups. Dopa-induced dyskinesia was observed in 96.8% of Group I and in 61.8% of Group II. As for progression of the disease, the Hoehn-Yahr stage of patients on medication was evaluated as 2.2 +/- 0.7 (mean +/- SD) in Group I and 3.1 +/- 1.1 in Group II for a period of 10 to 20 years of onset, 2.5 +/- 0.8 in Group I and 3.3 +/- 0.5 in Group II for 20 to 30 years, and 3.2 +/- 0.9 in Group I and 4.3 +/- 0.6 in Group II after 30 years. There were significant differences between the two groups in the frequency of positive family history, in the mean and distribution of age at onset, in the incidence of dystonic gait as the initial symptom, and in the incidences and medians of the variables including dystonia, hyperreflexia and dopa-induced dyskinesia, as well as in the progression of the disease. Thus, we have successfully characterized the clinical features of AR-EPDF and demonstrated that diurnal fluctuation is a cardinal symptom of this disease. Reported pathologic studies on AR-EPDF showed the nigral lesion characterized by non-Lewy body type degeneration and the occurrence of melanin-poor neurons. These pathologic findings as well as the clinical manifestations differentiate AR-EPDF from Parkinson's disease and from autosomal-dominant familial parkinsonism. Low melanin-content of the nigral neurons is also a striking feature of hereditary progressive dystonia with diurnal fluctuation, in which unlike AR-EPDF there is no neuronal loss in the substantia nigra.

Adult↗
PubMed1995

[Vasomotor impairment in patients with diabetic neuropathy--evaluation by laser Doppler flowmetry].

Using laser Doppler flowmetry, we evaluated cutaneous microcirculation in 28 patients with diabetic neuropathy and 18 control subjects. Skin blood flow in the hand was significantly reduced in the patients compared with that in age-matched controls. Skin blood flow in the hand of the patients correlated significantly with the amplitude of sensory nerve action potential of the median nerve. Significant correlation was shown in the patients between skin blood flow in the hand and changes in systolic blood pressure on standing from a supine position. However, there was no correlation between skin blood flow in the hand and variation in the R-R interval. These results suggest that the skin blood flow may decrease with the progression of diabetic neuropathy, particularly sympathetic autonomic neuropathy, and that neural control may be an important factor in the regulation of skin blood flow. Cold water immersion test revealed that there was no significant difference in vasoconstrictive response during the cold challenge, while recovery time of vasodilatory response after withdrawal of the thermal challenge was significantly prolonged in the patients as compared with that in the controls. In addition, it is suggested that the patients with a small increase or normal in skin blood flow of the hand may possess abnormalities predominantly in vasoconstrictive tone, whereas the patients with a decrease in skin blood flow of the hand in vasodilatory tone. Consequently, microcirculation in skin of extremities changes under the balance of vasoconstrictive and vasodilatory tones in patients with diabetic neuropathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Diabetic Neuropathies↗
PubMed1994

[Antiplatelet therapy in patients with cerebral thrombosis at the chronic phase--assessment of its effect on coagulation and fibrinolytic parameters].

We studied the effect of antiplatelet therapy not only on the secondary prevention of stroke but also on the suppression of vascular damages in patients with cerebral thrombosis at the chronic phase. We measured von Willebrand factor (vWF) as a marker for the endothelial system, and coagulation and fibrinolytic parameters in addition to platelet functions. The platelet aggregation and markers for platelet activation were monitored for the adequate inhibition of platelets. Twenty-one patients were treated with 200 mg ticlopidine. 9 patients with 100 mg ticlopidine and 60-150 mg acetylsalicylic acid, and 18 patients with 200 mg cilostazol daily. The mean duration of follow up was 8.4 +/- 3.0 months. A patient was attacked by a recurrent stroke, but no fatal vascular events occurred during the period. A significant decrease was observed in the collagen- and ADP-induced platelet aggregation and markers for platelet activation such as platelet factor 4 (PF4) and beta-thromboglobulin (beta TG) by the antiplatelet therapy. In addition, the activities of coagulation factor VIII (FVIII) and vWF, markers for vascular damages, showed a significant decrease. The results suggest that the antiplatelet therapy could ameliorate the vascular damage through the inhibition of platelet function. Moreover, thrombin-antithrombin III complex (TAT) and alpha 2-plasmin inhibitor-plasmin complex (PIC), markers for the activation of coagulation and fibrinolytic systems, decreased significantly, suggesting that the treatment inhibits the activation of coagulation and fibrinolytic systems induced by the platelet activation. The activities of FVIII and vWF decreased significantly when the level of beta TG or that of PF4 lowered sufficiently by the treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1993

[Early-onset parkinsonism with diurnal fluctuation--clinical and pathological studies].

The literature regarding parkinsonism of early-onset indicates that it encompasses several diseases differing in their clinical and pathological features. Since 1968 we have reported cases of early-onset parkinsonism with diurnal fluctuation of symptoms (abbrev. as EPDF). Attention has been focused on its similarities to or differences from Parkinson's disease (PD), juvenile parkinsonism of Yokochi, and hereditary progressive dystonia (HPD) of Segawa et al. In this paper we report the clinical and pathological characteristics of EPDF to facilitate its nosological identification. We examined the pathological features of EPDF in two cases. Case 1 was a 52-year-old female with consanguineously related parents and two other affected sisters. The disease began at the age of 20, and she had marked diurnal fluctuation of symptoms. With the assistance of L-dopa and bromocriptine, she was able to enjoy relatively satisfactory daily life activity until the age of 50. In the last two years she was bed-ridden with advanced parkinsonism. Case 2 was a 56-year-old man without any contributing family history. His disease started at age 26 and his symptoms showed slight fluctuation during the earlier stages of the disease. Treatment with L-dopa and bromocriptine was associated with marked up-and-down phenomenon and choreatic dyskinesia. Pathological study in the two cases revealed marked cell loss in the substantia nigra zona compacta, especially in the area A9, while the neuronal cell population of the ventral tegmental area (A10), locus caeruleus, superior raphae nucleus, and substantia innominata was relatively well preserved. There were no Lewy bodies.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗
PubMed1993

[Acrodystrophic neuropathy in an alcoholic].

The patient was a 48-year-old alcoholic man with no contributory family history. At age 36 he had developed sensory dominant polyneuropathy with highly impaired temperature sensation and deep sensation in the lower extremities, recurrent ulcers of the toes, and sexual impotence. A sural nerve biopsy at this time revealed marked loss of myelinated fibers with relative preservation of the population of unmyelinated fibers. Subsequently, he developed muscle atrophy of the lower thighs, urinary incontinence, and Wernicke's encephalopathy, and became non-ambulatory at age 44. The peripheral nerve conduction findings suggested predominantly axonal degeneration. The entire course was characterized by alternative progression and partial recovery influenced by his alcohol intake and nutritional state. Alcoholic neuropathy is a major cause of solitary acrodystrophic neuropathy (ADN). Manifestations of autonomic and motor neuropathy are more marked in alcoholic ADN than in HSAN-I, and central nervous system involvement is the hallmark of alcoholic ADN. In the treatment of patients with alcoholic ADN, attention should be paid to diabetes mellitus, malnutritional state, and vitamin deficiency, which frequently complicate alcoholism.

Alcoholism↗
PubMed1993

[Coagulation and fibrinolytic parameters in patients with various angiopathies--analysis in cerebral thrombosis, diabetic and vasculitic neuropathies].

We measured the coagulation and fibrinolytic parameters in patients with various angiopathies to clarify the usefulness of the parameters for the evaluation of vascular involvement. The study included 65 patients with cerebral thrombosis at the chronic stage, 47 diabetics with neuropathy associated with diabetic microangiopathy, 15 diabetics without neuropathy, and 16 patients with vasculitic neuropathy associated with collagen diseases. Control subjects were 45 patients with other neurological disorders without symptomatic angiopathies. In cerebral thrombosis at the chronic stage, the coagulation factor VIII (FVIII), von Willebrand factor (vWF), and platelet factor IV were elevated significantly as compared with age-matched controls. Diabetics with neuropathy showed significantly elevated FVIII and vWF in comparison with diabetics without neuropathy and controls. No significant difference in parameters concerning glucose metabolism was observed between the two groups of diabetics. These findings suggest that elevated activities of the coagulation parameters reflect not merely the abnormalities in glucose metabolism but also the presence of microangiopathy which causes diabetic neuropathy. The patients with vasculitic neuropathy exhibited significantly elevated FVIII, vWF and fibrin/fibrinogen degradation products (FDP). The present study demonstrated that FVIII and vWF are useful parameters for the evaluation of the extent of vascular involvement in angiopathies of different etiologies. It is important for the treatment of patients with the angiopathies, to monitor the coagulation parameters which reflect vascular involvement in addition to parameters for the disease activity.

Aged↗
PubMed1990

Autoimmune mechanisms in peripheral neuropathies.

In certain patients with demyelinating neuropathy and plasma cell dyscrasia, there are IgM monoclonal antibodies that recognize a carbohydrate epitope shared by myelin-associated glycoprotein (MAG) and at least two acidic glycolipids in the peripheral nervous system (PNS). The structures of the two acidic lipids have been elucidated as a new class of glycosphingolipids, termed sulfoglucuronyl glycolipids (SGGLs). SGGLs have been demonstrated to be present in myelin, axolemma, and other glia-related membranes in PNS of several animal species, as well as in human dorsal root ganglia and sympathetic ganglia. In rabbits sensitized with sulfoglucuronyl paragloboside (SGPG), a major SGGL in PNS, antibodies developed with reactivities toward SGPG and MAG. The animals also showed moderate weakness, a slowed nerve conduction velocity, and evidence of conduction block. Recently we also found SGPG in rat brain microvessels. This finding supports our hypothesis that autoantibodies may first interact with endothelial cell-bound antigens and that this might change the permeability of the blood-brain or blood-nerve barrier to permit the entry of these autoantibodies into the nervous system. Our data are consistent with the concept that an autoimmune response against the sulfoglucuronyl residue may participate in the pathogenesis of immune-mediated neuropathy.

Animals↗
PubMed1990

Sulfate incorporation into peripheral nerve endoneurial glycolipids after crush and permanent transection injury.

The sulfation of peripheral nerve glycolipids was examined at 35 days after both crush injury or permanent transection of the adult rat sciatic nerve by in vitro incorporation of [35S]sulfate into endoneurial slices. These experimental models of neuropathy are characterized by the presence and absence of both axonal regeneration and subsequent myelin assembly. Although the sulfo-glucuronosyl glycosphingolipids (SGGLs) were not detected by alpha-napthol reagent after HPTLC separation of the total acidic lipid extract, fluorographic analysis after sulfate incorporation revealed a 4.7-fold increase in [35S]sulfate in the sulfo-glucuronosyl paragloboside (SGPG) and a 3.5-fold increase in the sulfo-glucuronosyl-lactosaminosyl paragloboside (SGLPG) after the crush injury compared to permanent transection. These [35S]sulfate-labeled lipids were identified by comigration after HPTLC separation by immunostaining with specific IgM monoclonal antibodies from a patient with demyelinating neuropathy and plasma cell dyscrasia. Enhanced incorporation of sulfate in the crushed nerves was also observed in the sulfatides and in several unknown lipids migrating between GM2 and GM3, between GM1, and GM2, slightly above the origin, and at the origin. Since previous studies (Yao and Poduslo: J Neurochem 50:630-638, 1988) have shown [35S]sulfate incorporation, but not [3H]Gal or [3H]Glc, into sulfatides at 35 days after transection, it is possible that the sulfation observed in the present studies does not represent de novo biosynthesis but rather sulfation of an endogenous pool of glycolipids that results from the nerve injury.(ABSTRACT TRUNCATED AT 250 WORDS)

Amino Sugars↗
PubMed1990

Sulfated glucuronyl paragloboside in rat brain microvessels.

In patients with neuropathy associated with paraproteinemia, there are monoclonal immunoglobulin M antibodies reacting with myelin-associated glycoprotein and sulfated glucuronyl glycolipids. There are indications that the monoclonal antibodies may be responsible for these neuropathies. However, the mechanism by which the antibodies gain access to the nervous tissue, which is separated by the blood-brain barrier or blood-nerve barrier, is still unknown. In this study, we examined the presence of the sulfated glucuronyl glycolipid antigens on brain endothelial cells. Microvessels were isolated from adult Lewis rat brain cortex. Sulfated glucuronyl paragloboside (SGPG) was detected in the acidic lipid fraction by a TLC immunostaining method. Immunofluorescence studies showed positive staining on the surface of microvessels. In addition, SGPG could be detected in the cultured endothelial cells of human umbilical vein. These findings suggest that the endothelial cells contain antigenic sites for interaction with the autoantibodies. This type of interaction may result in damages to the endothelial cell function and may be responsible for changes in the blood-brain barrier permeability and the ensuing penetration of large molecules, such as immunoglobulins, into the endoneurial space.

Animals↗
PubMed1990

[Guillain-Barré syndrome and disturbance in multiple organs associated with cytomegalovirus infection].

We presented a case of Guillain-Barré syndrome (GBS) associated with pneumonitis, liver, pancreatic and renal dysfunction, and encephalopathy, who showed an increase in anti-cytomegalovirus (CMV) antibody titer of the serum and cerebrospinal fluid (CSF). A 41-year-old male admitted to our hospital with complaints of gait disturbance and muscle weakness in the extremities. The symptoms appeared following common cold, and developed gradually. Neurological examination showed mild mental symptoms, bilateral abducens, facial and bulbar palsy. Muscle weakness was shown on the neck and four extremities. Bilateral hand tremor, glove and stocking type sensory disturbance, and dysautonomia were also presented. Examination of the CSF revealed albumin-cytologic dissociation, and the anti-CMV antibody titer of the serum and CSF increased significantly. The titer of anti-CMV IgG antibody was 1:6400 in the serum, and was 1:80 in the CSF. Immunological examination showed no immunodeficiency. The conduction velocity of the peripheral motor and sensory nerves was decreased. The coefficient variation (CV%) of the R-R interval was also decreased. P1 and P2 latency and central conduction time for a somatosensory evoked potential (SEP) were also elongated, and paroxysmal abnormality was present on electroencephalogram (EEG). Two days after admission, pneumonitis appeared, leading to respiratory insufficiency. He needed respirator because of bulbar and respiratory disturbance. The chest roentgenogram revealed a consolidation-like shadow in the right upper lung filed. Two weeks after admission, liver, pancreatic and renal dysfunction was noted by biochemical examination of the blood, urinalysis, and abdominal echography. Since 10 days after the onset of GBS, 70 mg/day prednisolone was administered.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

Lymphocyte alpha-glucosidase in late-onset glycogenosis type II.

We describe the biochemical characterization of lymphocyte alpha-glucosidase in a 23-year-old man with intermediate clinical features between the childhood and adult forms of glycogenosis type II (Pompe's disease). Acid alpha-glucosidase activity was markedly reduced, but immunologic cross-reactive material against human liver acid alpha-glucosidase protein could be detected, and its amount was normal. In this patient, the disorder was induced by the catalytically inactive enzyme with a normal amount of enzyme protein.

Adult↗
PubMed1989

Partial characterization of leucocyte alpha-glucosidase in late onset glycogenosis type II.

We describe partial characterization and properties of leucocyte alpha-glucosidase from a patient with clinical features intermediate of juvenile and adult onset forms of glycogenosis type II. Acid and neutral alpha-glucosidase activities toward 4-methylumbelliferyl glucopyranoside as substrate were studied in total leucocytes, and separately in lymphocytes and granulocytes. Lymphocytes, which showed markedly reduced activities of acid alpha-glucosidase in the patient, are the most reliable peripheral blood cells for the diagnosis of glycogenosis type II. Moreover, the ratio of acid/neutral alpha-glucosidase activities, especially in lymphocytes, is a useful parameter for the diagnosis. In lymphocytes, the Km values of both acid and neutral alpha-glucosidases were essentially the same between the patient and normal controls; the Vmax value of acid alpha-glucosidase from the patient was markedly reduced, and the Vmax value of neutral alpha-glucosidase from the patient was reduced by 36% as compared with that from normal controls. Heat-inactivation experiments revealed that acid alpha-glucosidase activities of lymphocytes were relatively heat-stable, while both acid and neutral alpha-glucosidases of granulocytes were heat-labile. No differences in these properties, however, could be detected between the patient and normal controls.

Adult↗
PubMed1988

Antibodies to sulfated glucuronic acid containing glycosphingolipids in neuropathy associated with anti-MAG antibodies and in normal subjects.

Serum of patients with neuropathy and IgM monoclonal antibodies (M-proteins) that bind to the myelin-associated glycoprotein (MAG) were tested for binding to the major cross-reactive sulfated glucuronic acid containing glycosphingolipid, sulfated glucuronic acid paragloboside (SGPG). IgM binding to the glycolipid was detectable at serum dilutions of 1:10,000 and reactivity was greatest at 4 degrees C. Low titers of IgM binding to the glycolipid were also detected in sera from normal subjects and from patients with neurologic or rheumatologic diseases without serum M-proteins. Binding activity was present in 25% of the sera tested, and titers ranged between 1:25 and 1:400. One patient with peripheral neuropathy, however, had a measurable titer of 1:12,800 in the absence of monoclonal gammopathy. The study indicates that cold reacting anti-SGPG IgM antibodies are frequent constituents of the normal human antibody repertoire, and that monoclonal or polyclonal expansion of B cells that secrete these antibodies, is associated with peripheral neuropathy.

Antibodies, Monoclonal↗
PubMed1988

Preparation and characterization of antibodies against a sulfated glucuronic acid-containing glycosphingolipid.

In some patients with demyelinating neuropathy there are immunoglobulin M paraproteins that react with carbohydrate determinants shared by myelin-associated glycoprotein (MAG) and two peripheral nerve acidic glycolipids, termed sulfoglucuronosylglycosphingolipids (SGGLs). To study the antigenicity of these glycolipids, we immunized three New Zealand white rabbits with sulfoglucuronosylparagloboside (SGPG), a major SGGL in peripheral nerve, emulsified in Freund's complete adjuvant and keyhole limpet hemocyanin. All three rabbits inoculated with SGPG showed weight loss and mild weakness, predominantly in their hind feet, 2-5 weeks postinoculation (PI). Two of the three rabbits again showed moderate weakness 3 and 8 months PI, respectively. Electrophysiological studies demonstrated a slowed nerve conduction velocity in the sciatic nerve. Anti-SGPG antibody titers in sera were detected at dilutions of 1:1,000 to 1:2,500 by an enzyme-linked immunosorbent assay. Although all three rabbit sera reacted with SGGLs, two reacted with a desulfated form of SGPG and the other did not, suggesting a fine heterogeneity in antigenic specificity. As with sera from patients with demyelinative paraproteinemia, all rabbit sera reacted with MAG in human CNS and PNS myelin. They also reacted with MAG from bovine CNS myelin as well as several low-molecular-weight glycoproteins in bovine peripheral nerve myelin. Thus, we demonstrated that the rabbit antisera generated against SGPG have the same or similar antigenic specificity as those of the anti-MAG M-proteins from patients with neuropathy. The results suggest that an autoimmune response against the sulfoglucuronosyl residue may participate in the immunopathogenesis of this type of neuropathy.

Animals↗