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Query: UMLS:C0011854 (type 1 diabetes)
 20,749 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Only a few studies have investigated vestibular function in diabetes mellitus (DM), showing contradictory results. We have performed an electronystagmographic (ENG) evaluation of 46 individuals with type I DM and 37 healthy controls. No patient reported subjective vestibular symptoms. Duration of caloric-induced nystagmus (DN) was significantly lower (2.1 +/- 0.7 vs. 2.6 +/- 0.4 min, p less than 0.01), and central nystagmus frequency of caloric response also nonsignificantly tended to be decreased (37.4 +/- 16.5 vs. 41.7 +/- 12.7 beats/30s, p = 0.21) in DM patients, as compared to controls. The latter comparison achieved significance after exclusion of newly diagnosed diabetic patients (33.4 +/- 16.1 vs. 41.6 +/- 12.7 beats/s, p less than 0.05). Depressed caloric reactions were seen in 21.8% of patients. DN was lower in patients with microalbuminuria and retinopathy, but this was not observed after exclusion of newly diagnosed diabetic patients, all of whom had normal ENG responses and no chronic diabetic complications. The existence of a lower DN and central nystagmus frequency should be borne in mind when interpreting ENG tracings in patients with long duration type I diabetes mellitus.
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PMID:Asymptomatic electronystagmographic abnormalities in patients with type I diabetes mellitus. 178 72

The authors report on one case of Wolfram syndrome, a rare condition, which is characterized by juvenile onset diabetes mellitus, diabetes insipidus, optic atrophy and sensorineural deafness. The findings of this 13-year follow-up show that this patient developed typical neurological complications of long-standing diabetes mellitus as in the common type 1 variant. Moreover, some peculiar signs occurred such as anosmia, ophthalmoplegia interna, and central nystagmus. Since Wolfram syndrome is probably part of a more generalized neurodegenerative disorder, long-term prognosis will depend both upon the severity of chronic diabetic complications and upon the rapidity, by which degeneration of cerebellar, pontine and brain stem structures appear. Prognosis of the cardinal clinical signs is such that optic atrophy, though usually quite rapid in the beginning, generally does not lead to complete blindness. Sensorineural hearing loss progresses very slowly so that deafness might be expected exceptionally only. The hearing deficit in classical diabetics, however, is of retrocochlear origin. Therefore, in Wolfram syndrome, a combined inner-ear and retrocochlear hearing loss may occur.
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PMID:Juvenile onset diabetes mellitus, central diabetes insipidus and optic atrophy (Wolfram syndrome)--neurological findings and prognostic implications. 185 94

The association of diabetes mellitus, optic nerve atrophy, and sensorineural deafness is a rare but distinct syndrome. We describe an 18-year-old woman who developed diabetes mellitus at three years of age, sensorineural deafness at 14 years of age, and bilateral asymptomatic optic atrophy at 18 years of age. Diabetic retinopathy was notably absent. Forty-two patients with diabetes mellitus and optic atrophy have been described. All had type I diabetes mellitus without typical diabetic retinopathy even though insulin-dependent diabetes mellitus was present for many years. Other neurologic abnormalities, including diabetes insipidus, epilepsy, and nystagmus, have been described in association with diabetes mellitus and optic atrophy. A distinct, heredofamilial genetic mechanism, unrelated to diabetes mellitus per se, is postulated.
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PMID:Coexistence of diabetes mellitus, optic atrophy, and sensorineural deafness: case report. 371 43

Wolfram's syndrome, also known as DIDMOAD syndrome, includes juvenile diabetes mellitus and optic atrophy variously associated with diabetes insipidus and deafness. We describe the neurological findings in 5 patients with Wolfram's syndrome. All patients had a neurological examination and were subjected electrophysiological and brain imaging including CT scan and, in one patient, MRI. There were two pairs of brothers and a sporadic case with paternal consanguinity suggesting recessive inheritance. Neurological abnormalities were found in four patients including dysarthria, seizures, anosmia, nystagmus, ataxia and changes in the electroencephalograms, electroretinograms and evoked potentials. In contrast with previous reports, four patients had abnormal brain CT scan with prominent atrophy of the brainstem. In the patient studied with NMR, severe brainstem and cerebellar atrophy was found. These neuroradiological findings are reminiscent of those described in olivopontocerebellar atrophy and are in agreement with previous pathological studies. We conclude that Wolfram's syndrome includes phenotypical manifestations of olivopontocerebellar atrophy. This reinforces the opinion that olivopontocerebellar atrophy is a nonspecific syndrome of varied causes.
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PMID:[Neurologic manifestations in Wolfram's syndrome]. 833 58

The Wolfram syndrome is a rare dysmorphogenetic disease of autosomic recessive hereditary nature. The pathogenesis of the disease is still not well known. It is characterised by the presence of diabetes insipidus, diabetes mellitus, optic atrophy and deafness. Other anomalies, such as renal outflow tracts and multiple neurological disorders may develop later. In our case report the diabetes mellitus appeared at the age of 4; the hearing loss and renal disturbances at the age of 11; the optic atrophy at the age of 16. No signs of ataxia, diabetes insipidus and neurologic anomalies were found. The diagnosis of Wolfram syndrome is not always easy in the first stages of the disease. The suspect may come from the presence of a juvenile diabetes mellitus asssociated with optic atrophy. For the diagnosis a valid clue can be given from the results of some clinical tests such as the positivity of the visual evoked potentials and the retinogram reliefs and the exclusion of the autoimmune origin of the diabetes mellitus. Other signs such as the progressive sensorineural hearing loss, the presence of nystagmus and of urodynamic disturbances and renal complications makes the diagnosis of this syndrome easier.
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PMID:Wolfram syndrome. 1472 94

A 24-year-old female who was recently diagnosed with Type 1 diabetes mellitus (TiD) presented with a five-year history of visible gait disturbance and slurred speech. Her neurologic examination was remarkable for dysarthria, bilateral nystagmus, dysdiadochokinesia, finger-nose incoordination, heel-knee incoordination, and ataxic gait. A brain MRI disclosed diffuse cerebellar atrophy. Her serum antiglutamic acid decarboxylase (GAD) antibody titer was elevated. Antinuclear antibody (ANA) test was positive with atiterofl:2560 and a speckledpattern. Genetictests for inherited ataxia, including Friedreich ataxia, were negative for mutations. Her cerebrospinal fluid (CSF) analysis revealed oligoclonal bands and she had a positive CSF GAD65 antibody. A diag- nosis of GAD antibody-induced cerebellar ataxia was considered. She developed GAD autoimmune antibody positive TiD during the course ofher dis- ease. GAD antibody-associated cerebellar ataxia is a rare entity, however it should be considered as a possibility in patients with associated autoimmune disease and positive anti-GAD antibody.
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PMID:High Titer of Circulating Antiglutamic Acid Decarboxylase Antibodies in a Patient with Cerebellar Ataxia and Type 1 Diabetes. 2977 59