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Biomedical subjects

Y Harati

Publications and source records attributed to Y Harati.

At least 55 records · Page 3Linked to original sources
PubMed1986

Childhood dermatomyositis in monozygotic twins.

Identical twins who developed childhood dermatomyositis 2 weeks apart following upper respiratory infections are reported. Observations suggest that genetic influence on host susceptibility may play a role in childhood dermatomyositis and that a combination of host and environmental factors is most likely necessary to produce the disease.

Autoimmune Diseases↗
PubMed1986

Clinical significance of selective nerve fascicular degeneration on sural nerve biopsy specimen.

Eight cases of selective nerve fascicular degeneration (SNFD) on sural nerve were identified from 250 consecutive biopsy specimens. In six patients, SNFD was associated with angiopathic changes. This study suggests that SNFD is a distinctive finding seen in 3% of nerve biopsy specimens, and its presence alone may indicate an angiopathic disorder. Selective nerve fascicular degeneration is associated with a variety of clinical presentations, ranging from chronic distal symmetrical polyneuropathy to mononeuropathy multiplex.

Adult↗
PubMed1986

Silent myocardial infarction and diabetic cardiovascular autonomic neuropathy.

Seventy-three consecutive diabetic adults with symptomatic peripheral neuropathy were evaluated for the presence of cardiovascular autonomic neuropathy and electrocardiographic evidence of myocardial infarction (MI). Twenty-five (34.2%) patients demonstrated cardiovascular autonomic neuropathy, and ten (13.7%) patients had electrocardiographic evidence of MI. Of the ten MI identified, seven were asymptomatic (silent) by history. The incidence of silent MI was significantly higher (P less than .04) in patients with cardiovascular autonomic neuropathy. It is postulated that sudden death in diabetic patients with cardiovascular autonomic neuropathy may be due to silent MI.

Adult↗
PubMed1985

Congenital hypomyelinating neuropathy.

Two patients with congenital hypomyelinating neuropathy are reported with details of sural nerve pathology. The resemblance of this condition to the hypomyelinating neuropathy of Trembler mice is discussed and the pertinent medical literature reviewed.

Axons↗
PubMed1985

Tubular aggregates: their association with myalgia.

Three thousand consecutive muscle biopsies were reviewed for the presence of tubular aggregates and their association with clinical symptomatology. Tubular aggregates were detected in 19 patients (0.6%). Twelve of these nineteen patients had severe myalgia, and the most abundant tubular aggregates were found in biopsies of patients with myalgia. Seven patients had only myalgia as their clinical symptomatology with normal physical examination. An additional five patients with tubular aggregates and myalgia had concomitant amyotrophic lateral sclerosis (2) or neuropathy (3). The high incidence of myalgia associated with tubular aggregates in our patients and the fact that tubular aggregates originate from sarcoplasmic reticulum suggest a role played by this structure in the pathogenesis of myalgia.

Amyotrophic Lateral Sclerosis↗
PubMed1984

Sialosylglobotetraosylceramide: a marker for amyotropic lateral sclerosis.

Gangliosides of healthy and pathologic muscles (amyotropic lateral sclerosis and facio-scapulo-humeral muscular dystrophy) were studied. Total ganglioside content of the affected muscles was approximately 2 fold higher than the unaffected muscles. Our results showed that ALS muscle contained a ganglioside which was absent in the unaffected and FSH muscular dystrophic muscles. Based on the results of hydrolysis with Vibrio cholerae neuraminidase and subsequent reactivity of the asialo derivative towards anti-globotetraosylceramide, we propose that the ALS ganglioside is sialosylglobtetraosylceramide, NeuAc(alpha 2-3)Ga1NAc(beta 1-3)Ga1(alpha 1-4)Ga1(beta 1-4)G1c-Cer.

Amyotrophic Lateral Sclerosis↗
PubMed1984

Coronary artery disease in patients with cerebrovascular disease: a prospective study.

Coronary artery disease is the cause of death in most patients who have transient ischemic attacks or stroke. Evaluation for this condition is not routinely performed in such patients, and no prospective studies have been reported. We prospectively examined 50 consecutive patients with transient ischemic attacks or mild stroke to determine the prevalence and importance of coronary artery disease. All patients were examined by a cardiologist and underwent both exercise thallium-201 scintigraphy and exercise radionuclide ventriculography. Sixteen patients were suspected to have coronary artery disease on the basis of clinical evaluation. In 15 of these the was confirmed by the nuclear scans. The remaining 34 patients had no clinical evidence of heart disease, yet 14 had abnormal cardiac scans. Twenty of 22 patients with abnormal scans who underwent cardiac catheterization had significant coronary artery disease or a cardiomyopathy. The discovery of heart disease altered clinical management in 13 patients. Overall, 29 of 50 patients had significant coronary artery disease, compared with a 7% prevalence of the condition in other patients of similar age at the same institution.

Cerebral Angiography↗
PubMed1984

Adult onset idiopathic familial brain calcifications.

We treated a family with idiopathic calcifications of symmetric areas of the brain, including the basal ganglia, dentate, and cerebral white matter. Dementia, progressive dysarthria, incontinence, propulsive-ataxic gait, fixed facies, and cogwheel rigidity without dysmorphic features develop in affected persons. Calcium, phosphorus, and parathyroid hormone levels were normal in the two siblings tested. The literature is reviewed and five other families with a similar syndrome are identified. These six families seem to be clinically distinct from the larger group of idiopathic cerebral calcifications usually referred to as Fahr's disease.

Adult↗
PubMed1983

A dominantly inherited syndrome with continuous motor neuron discharges.

A distinct syndrome with continuous motor neuron discharges apparently developed in seven members of a single family, involving both sexes and spanning three generations. Persistent vermiform twitching and episodic stiffness predominantly in lower extremity muscles occurred in early childhood and tended to be less severe in adulthood. In 2 patients the clinical manifestations improved with oral phenytoin and carbamazepine but not with parenteral diazepam. Insertional activity was normal, and continuous, rhythmical, normal-appearing muscle discharges were observed on electromyography. The cerebrospinal fluid levels of homovanillic acid and 5-hydroxyindoleacetic acid were increased in the proband. The disappearance of continuous muscle discharges during spinal anesthesia and the lack of response to diazepam indicated generation of the discharges from the proximal portion of the motor unit.

Adult↗
PubMed1982

Evaluation of muscle degeneration in inherited muscular dystrophy by nuclear magnetic resonance techniques.

Nuclear magnetic resonance (NMR) techniques were applied to study the muscular dystrophy in chicks. The water proton spin-lattice relaxation times (T1) of fast, slow, and mixed muscles and plasma were measured. The T1 values of dystrophic pectoralis major and posterior latissimus dorsi (PLD) were significantly higher than those of the normal pectoralis and PLD muscles. The present results establish a direct relationship between the differences in T1 values and the severity of muscle degeneration. Consistent with this conclusion, it was also found that the T1 values of muscles unaffected in muscular dystrophy, namely, the gastrocnemius, and anterior latissimus dorsi (ALD), were not different between the normal and dystrophic chicks. Although the affected muscles of dystrophic chicks contained higher percent water and fat than those of normal chicks, the results show that the higher T1 values in dystrophic muscles were not solely due to variations in their water content. The increase in the T1 values is principally a result of altered interaction between cellular water and macromolecules in the diseased muscles. These data also point out the potential use of NMR imaging in evaluating muscle degeneration.

Adipose Tissue↗
PubMed1980

Central core disease and malignant hyperthermia syndrome.

In a detailed investigation of a family with musculoskeletal abnormalities observed in four generations, the proband and his mother were found to have central cores and multicores on histochemical and electron microscopic studies of biopsied muscle. A male sibling experienced a malignant hyperthermic reaction during hand surgery, and although similar reactions had not been observed in other family members, unexplained ventricular dysrhythmias did occur in the proband during surgery. The proband and his mother were subsequently shown to be susceptible to malignant hyperthermia by abnormal in vitro muscle contractures in the presence of halothane or caffeine. Physicians and dentists caring for patients with central core disease should be aware of its possible association with malignant hyperthermia. Complete evaluation of patients with central core disease should include in vitro muscle contracture studies for malignant hyperthermia since many of patients require surgery for musculoskeletal defects.

Child↗
PubMed1979

Prednisone use in concurrent autoimmune diseases.

A 40-year-old woman with clinical and laboratory features of myasthenia gravis, hyperthyroidism, and polymyositis responded to treatment with prednisone alone. Symptoms of myasthenia gravis appeared first followed by hyperthyroid symptoms. Triiodothyronine, thyroxine, and thyroid uptake were elevated as were serum levels of CPK, SGOT, SGPT, and LDH. Muscle biopsy specimen showed mild type II fiber atrophy and a small focus of inflammatory cells. Two weeks after initiation of prednisone, 100 mg every other day, the ESR declined from 44 to 12 mm/hr, serum enzyme values became normal, and the weakness improved. Over the ensuing four months, the thyroid function values returned to normal and the patient no longer needed any anticholinesterase drug. At present, she is functionally normal except for mild defects in eye movement and she takes no medication. Physicians should consider treating patients who have several concurrent autoimmune diseases with prednisone to see if all conditions can be brought under control with one simple therapy.

Adult↗