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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Friedreich's ataxia (FA) is one of the genetic syndromes sometimes associated with diabetes and the most common hereditary ataxia. It is a autosomal recessive neurodegenerative disease, caused by a mutation in the FRDA gene, which originates decreased expression of frataxin, a mitochondrial protein involved in iron metabolism. The disorder is usually manifest in childhood and is characterised by ataxia, dysarthria, scoliosis and feet deformity. About two thirds of patients have hypertrophic cardiomyopathy, 10% have diabetes and 20% have another glucose homeostasis disorder. Both insulin resistance and beta-cell dysfunction are implicated in this patients' diabetes pathophysiology. The mean half-life is 35 years. Cause of death is usually related to cardiomyopathy or diabetes' complications. We report the case study of two twin sisters with 28 years old, in whom FA was diagnosed in the first decade, both of them with diabetes since their early twenties. A third sister with FA is reported, with no glucose homeostasis disorder. They also have two healthy male brothers. Based in this cases, the FA associated diabetes pathophysiology is discussed, concerning the therapeutic approach to these patients and to their diabetic relatives without neurologic symptoms. The role of molecular genetic testing and genetic counselling are also debated.
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PMID:[Friedreich ataxia and diabetes mellitus--family study]. 1668 89

A 60-year-old woman with a history of chronic back pain presented to the emergency department with headache, slurred speech, and altered sensorium reported by her family. The previous day, she had a lumbar catheter placed for symptomatic relief of her chronic back pain. The patient complained only of headache, but otherwise thought she was unaffected. The patient's past medi- cal history was remarkable for diabetes, hypertension, peripheral neuropathy, gastritis, supraventricular tachycardia, and chronic back pain. On physical examination she was alert, fully orientated, and in no acute distress. Her vital signs were normal. Neurological examination revealed subtle word-finding difficulties and dysarthria. There were no physical signs of raised intracranial pressure (ICP). The remainder of her examination was entirely normal.
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PMID:Pneumocephalus secondary to lumbar catheterization. 1696 Feb 96

We explored the manifestations of an autosomal-recessive multisystemic disorder in several Saudi families. Recognized causes of progressive extra-pyramidal disorder and white matter disease were excluded and the neurological, imaging, endocrine, and skin manifestations of this syndrome described. The onset of these symptoms in these patients began in early adolescence and progressed more rapidly in males. All affected patients had total or partial alopecia, clinical and chemical evidence of hypogonadism (low levels of estradiol and testosterone); females had clear evidence of hypogonadism (streak or absent ovaries), and some patients had diabetes mellitus and/or sensorineural deafness. The constant biochemical abnormality was the low IGF-1. The neurological manifestations included moderate to severe intellectual decline and abnormality of muscle tone and posture with choreo-athetoid and dystonic movements resulting in gait difficulty, dysarthria, difficulty swallowing, and scoliosis. The MRI of brain demonstrated white matter involving cerebellum, brain stem, and cerebral structures, as well as abnormal decreased signal intensity in the basal ganglia with involvement of the substantia nigra. We conclude that the association of hypogonadism, alopecia, and persistent low IGF-1 is a significant autosomal recessive syndrome; it is prevalent in Saudi Arabia. We also demonstrate that the progressive extra-pyramidal disorder, white matter disease, and abnormal signals of the basal ganglia are common features of this syndrome. Sensorineural deafness and diabetes mellitus were recognized features.
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PMID:Autosomal-recessive syndrome with alopecia, hypogonadism, progressive extra-pyramidal disorder, white matter disease, sensory neural deafness, diabetes mellitus, and low IGF1. 1716 99

We sought to determine the clinical pictures, topography and pathogenesis of patients with unilateral single or multiple corona radiata infarcts. We defined corona radiata ischemic stroke if the patient had a focal neurological deficit and a relevant non-hemorrhagic infarction confined to the vascular territory of a long medullary artery proved by CT and MRI with contrast. We compared risk factors and clinical feature between subtypes of corona radiata infarcts. The study sample represents 1.2% of the patients (68/5500) with first-ever stroke in our Registry, including ischemic and hemorrhagic strokes. There were 37 patients (54%) with single infarct presenting 17 different complete or partial sensory-motor symptoms with dysarthria; 14 patients (21%) with unilateral multiple infarcts in one hemisphere had often complete sensorimotor deficits with some neuropsychological impairment; among 17 patients (25%) with multiple infarcts in both hemisphere, one half had bilateral motor and sensory symptoms, and neuropsychological deficits, visual field defects were uncommon. Seventeen patients (25%) had dysarthria, which was no localizing value. Hypertension was the most frequent vascular risk factor in 62% of patients, smoking in 28%, diabetes mellitus in 26%, hypercholesterolemia in 9%, and atrial fibrillation in 7%. The main cause of corona radiata infarcts was small-artery disease with long-standing hypertension in 40 patients (59%), large-artery disease in 19%, cardioembolism in 12%. Most of the patients (88%) had leukoaraiosis, and patients with bilateral multiple infarcts, leukoaraiosis was more frequent than in those with unilateral single infarct (P = .016; < .05). Despite clinical similarity to that found in superficial and deep infarcts, incomplete motor and sensory symptoms and MRI allows to delineate simultaneous uni- or multiple infarcts in the corona radiata. After an acute and immediate onset, outcome at 6 months of stroke onset is in general benign except those with bilateral infarcts. The coexistence of small-artery disease with leukoaraiosis suggest similar vascular risk factors and physiopathological mechanism.
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PMID:Spectrum of single and multiple corona radiata infarcts: clinical/MRI correlations. 1790 7

Diabetes mellitus develops in about 10% of acromegalic patients, usually secondary to insulin resistance caused by growth hormone excess. Diabetic ketoacidosis is a result of relative insulin deficiency and is a rare feature of acromegaly. Here, we present one case of this disorder. A 57-year-old man came to the emergency room due to 2 weeks of dizziness. He also had polyuria, polydipsia, nausea, diplopia, blurred vision and dysarthria. His plasma glucose level was 32.06 mmol/L, plasma osmolarity was 322 mOsm/L, arterial pH was 7.30, level of bicarbonates was 18 mmol/L, urine ketones was 4+, and HbA1c was 14.1%. No specific cause for the development of this metabolic derangement could be found. He displayed clinical features of acromegaly during admission, which was confirmed by an elevated growth hormone level and pituitary macroadenoma shown on magnetic resonance imaging. The patient underwent total transsphenoid tumor removal 2 weeks later; plasma glucose levels became normal thereafter.
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PMID:Diabetic ketoacidosis in a patient with acromegaly. 1790 70

A 61-year-old woman with diabetes mellitus was admitted to our hospital with right hemiparesis and dysarthria. Brain MRI showed bilateral cerebral peduncular infarctions. Three days after admission, she was unable to generate any voluntary movements, except for those of the eye, suggesting locked-in syndrome (LIS). She could not speak, but showed good comprehension by blinking in response to verbal commands. Brain CT 5 days later revealed subarachnoid hemorrhage (SAH) around quadrigeminal and ambient cistern. Cerebral angiogram on the following day revealed no aneurysm, occlusion of right persistent primitive trigeminal artery (PPTA) and a little flow of the bilateral vertebral arteries. Eye movements were impossible in all directions on the 11th day and MRI showed new infarctions of the midbrain and the ventral portion of the pons. However, an EEG on the 20th day was almost normal. We speculated that low blood flow in the basilar artery from the PPTA caused bilateral cerebral peduncular infarctions, and that weakness of the PPTA caused SAH.
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PMID:[Locked-in syndrome due to bilateral cerebral peduncular infarctions with occlusion of persistent primitive trigeminal artery]. 1801 21

Woodhouse Sakati syndrome is a rare autosomal recessive neuroendocrine disorder characterized by the combination of alopecia, hypogonadism, diabetes mellitus, mental retardation, sensory neural deafness and extrapyramidal features. Movement disorders mainly consist of dystonia and chorea of the limbs with onset in adolescence. Facial muscles are usually spared, but dysarthria is common. Pyramidal features and peripheral abnormalities are inconsistent features. Most of the reported families are from the Middle Eastern countries although rarely Caucasian cases have been described. Here we present clinical details of two affected siblings from a new Middle East family and draw attention of movement disorder specialists to this entity. We summarize findings from pervious cases with particular focus on neurological and movement disorder features.
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PMID:Dystonia in the Woodhouse Sakati syndrome: A new family and literature review. 1817 54

Acute movement disorder associated with reversible bilateral basal ganglia lesions is an increasingly recognized syndrome in patients with end-stage renal disease, especially in the setting of concurrent diabetes mellitus. We report an elderly man with end-stage diabetic nephropathy treated by daily automated peritoneal dialysis who developed subacute symptoms of gait disturbance, dysarthria, dysphagia and lethargy. Computed tomography and magnetic resonance imaging of the head revealed bilateral symmetrical basal ganglia lesions. Repeat imaging 3 weeks later showed that these lesions had regressed spontaneously. However, his neurological symptoms improved slowly. These findings were similar to 23 other cases in the literature. Review of these cases shows that clinical features were predominantly bradykinesia, gait disturbance and concurrent metabolic acidosis (observed in 90% of cases). The pathogenesis of this condition has not been clearly defined, but uraemia may be an aggravating factor in predisposed patients, particularly in the presence of diabetic microvascular disease. There is no specific treatment for this condition; supportive measures are the mainstay of management. In the majority of patients, neurological improvement lags behind regression of basal ganglia lesions seen with neuroimaging, and the long-term outcome is variable.
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PMID:Bilateral basal ganglia lesions in patients with end-stage diabetic nephropathy. 1819 7

The syndrome of acute bilateral basal ganglia lesions presents with parkinsonism, altered mental status, dysarthria, and dysphagia in association with specific imaging findings in the basal ganglia. It is an uncommon syndrome seen almost exclusively in patients with diabetes mellitus and renal failure. Previously reported cases have all run a monophasic course, but we report a patient with a relapsing, remitting course. This 64-year-old diabetic man with uremia on hemodialysis had an acute episode of disordered sensorium. Brain computed tomography showed the classic findings of hypointensity of bilateral basal ganglia. He recovered from the episode, but had another with parkinsonian symptoms about 18 months later. Sequential brain images demonstrated encephalomalacia of the basal ganglia. His condition waxed and waned several times, but he eventually died of unknown causes less than 2 years after the first event. This syndrome, therefore, may not be limited to just one episode.
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PMID:The syndrome of bilateral basal ganglia lesions in diabetic uremic patients presenting with a relapsing and remitting course: a case report. 1822 16

Antemortem diagnosis of isolated inner ear infarction remains a diagnostic challenge since current imaging techniques do not permit differentiation of isolated labyrinthine infarction from other more benign disorders involving the inner ear. An 81-year-old woman with diabetes, hypertension and hypercholesterolemia presented with vertigo and hearing loss. Initial examination revealed findings of left cochleovestibular labyrinthitis without brainstem or cerebellar signs. MRIs including the diffusion-weighted images were normal. Five days later, however, she developed dysarthria, and decreased facial sensation, subtle facial palsy, and dysmetria in the left side. Follow-up MRI was consistent with acute infarctions in the territory of anterior inferior cerebellar artery (AICA), involving the left lateral pons, left middle cerebellar peduncle, and inferolateral cerebellum. AICA infarction should be considered in acute audiovestibular syndrome, especially in aged patients with vascular risk factors, even though the classic brainstem or cerebellar signs are absent.
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PMID:Progression of acute cochleovestibulopathy into anterior inferior cerebellar artery infarction. 1910 52


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