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Biomedical subjects

N Gadoth

Publications and source records attributed to N Gadoth.

At least 109 records · Page 6Linked to original sources
PubMed1989

[Syringomyelic form of leprosy].

A 60-year-old man suffered from recurrent painless burns. During his first neurological work-up, ulcers with trophic changes were noted on the dorsum of both hands. Temperature and pain sensation were decreased over the upper extremities, but touch, vibration and position sense were normal. Syringomyelia was considered, but neuroradiologic evaluation was negative. A year later he was readmitted and was found to have patchy anesthesia involving all extremities, but no distal muscle weakness or wasting; tendon reflexes were normal. Leprosy was diagnosed and confirmed by skin and nerve biopsies. This syringomyelic form of leprosy is difficult to diagnose as it often mimics intraspinal space-occupying lesions. In Israel, where leprosy is still endemic, unusual sensory disturbances, such as patchy dysesthesia, should suggest its possibility.

Diagnosis, Differential↗
PubMed1988

Autoimmune diseases other than lupus share common anti-DNA idiotypes.

We examined the sera of 170 patients with various autoimmune diseases other than systemic lupus erythematosus (SLE) for the presence of an anti-DNA antibody idiotype termed 16/6 and known to occur with high frequency in sera of patients with SLE. The idiotype was found in 6/15 sera from patients with polymyositis (49%), 3/18 with multiple sclerosis (17%), 3/18 with primary Sjögren's syndrome (18%), 9/40 with autoimmune thyroid diseases (23%), 2/35 with myasthenia gravis (6%), and 3/42 patients with rheumatoid arthritis (7%). The idiotype was not detected among 12 patients with scleroderma or 77 normal controls. The presence of the 16/6 idiotype was associated with the presence of another anti-DNA idiotype termed 134-Id. Serum samples were also tested for activity against DNA, various synthetic polynucleotides, and cardiolipin. The serum activity against these antigens was found to be polyspecific, though overlap in reaction against the various polynucleotides was not absolute. The 16/6 idiotype is thought to be coded by a germline gene. The presence of this idiotype in various autoimmune diseases points to a pathophysiologic link between the diseases.

Autoantibodies↗
PubMed1988

Electromyographic abnormalities in patients undergoing long-term therapy with etretinate.

Neuromuscular evaluation including electromyographic studies and serum muscle enzyme level determinations were performed in 13 patients who had been receiving etretinate for 1 to 2 1/2 years. None of the patients had complained of muscle pains or weakness. In 3 of 13 patients the electromyographic studies showed low amplitude and short duration action potentials. Mild muscular weakness was detected in one of these three. The levels of serum muscle enzymes were within the normal range in all patients. Results suggest that etretinate may cause subclinical muscle damage. In view of our results and previous reports, it seems that neuromuscular evaluation should be part of the routine follow-up of patients receiving long-term therapy with etretinate.

Action Potentials↗
PubMed1988

Abnormal orbicularis oculi reflex response in sleep apnea secondary to acromegaly. Evidence of pontomedullary dysfunction in sleep apnea syndrome.

Severe sleep apnea was present in a patient with upper airway obstruction due to acromegaly. The study of orbicularis oculi reflex responses (OORR) disclosed a marked prolongation of the late response prior to tracheostomy. Following the surgical relief of upper airway obstruction, sleep apnea disappeared, and the latency of the late response of the OORR was dramatically reduced but failed to normalize. The OORR and especially its late response were normal in a patient with acromegaly who did not experience sleep apnea. In two patients with sleep apnea, but without acromegaly, the late responses of the OORR were abnormal. It is suggested that the presence of abnormal OORR in sleep apnea may reflect a basic defect in pontomedullary control of respiration during sleep.

Acromegaly↗
PubMed1987

Evaluation of renal angiomyolipoma by traditional and modern imaging in a case of tuberous sclerosis.

Renal angiomyolipoma is known to affect mainly patients with tuberous sclerosis, although it can be found in a significant number of otherwise normal individuals. Few cases have been published in which angiomyolipoma and renal cell carcinoma occurred in the same kidney. In such cases the radiologist may be asked if it is possible to distinguish between the two tumors in the same kidney. The present case report describes systemic traditional and modern imaging of huge bilateral angiomyolipoma in tuberous sclerosis. The analysis of the data implies that, although the diagnosis of angiomyolipoma can be established with noninvasive techniques only, it is impossible to distinguish between the two mentioned tumors if they coexist in the same kidney, even if all available imaging techniques are used.

Adult↗
PubMed1987

Epilepsy among parents of children with cleft lip and palate.

It is well established that children of mothers with epilepsy may suffer from facial clefts more frequently than children of non-epileptic mothers. However, a wide range of prevalence rates in this particular population is quoted in the literature. The majority of studies were performed by screening epileptic mothers for the presence of facial clefts in their offspring. The present study screened a relatively large group of children with primary facial clefts and established the prevalence rate of epilepsy among their mothers. It was found that the frequency of epilepsy among Jewish Israeli mothers to children with facial clefts is four times higher and the frequency of facial clefts in children of epileptic mothers is six times higher than in the respective normal population.

Abnormalities, Drug-Induced↗
PubMed1987

Episodic hormone secretion during sleep in Kleine-Levin syndrome: evidence for hypothalamic dysfunction.

"Acute" hypothalamic-pituitary function tests including insulin tolerance test, LRH, ACTH and TRH stimulation tests and nocturnal secretory pattern of human growth hormone, 11-OHCS, prolactin, FSH, LH and TSH were studied in a 23-year-old male with Kleine-Levin syndrome during the course of a typical hypersomnic attack. The "acute" tests revealed paradoxical growth-hormone response to TRH stimulation, borderline high basal plasma prolactin levels with normal response to TRH. The hormonal secretory pattern during sleep revealed abnormalities in LH, 11-OHCS and prolactin secretion. These together with the results of the "acute" tests are indicative of an abnormality in the hypothalamic regulation of various pituitary hormones. This observation may indeed be the first laboratory demonstration confirming a long-standing hypothesis that Kleine-Levin syndrome is related to hypothalamic dysfunction.

Adult↗
PubMed1987

Etretinate-induced skeletal muscle damage.

Three patients who received etretinate, two for psoriasis vulgaris and one for exfoliative dermatitis, developed clinical and electromyographic features of muscle damage during treatment. In one patient histological and ultrastructural findings indicated segmental muscle necrosis. Withdrawal of the drug led to clinical recovery and normalization of muscle enzyme levels and electromyogram. To the best of our knowledge, this is the first report to show etretinate-induced reversible skeletal muscle damage.

Adult↗
PubMed1987

Fluctuating dystonia responsive to levodopa.

Four cases of hereditary progressive dystonia with diurnal fluctuation were studied. All were sporadic; three of them mimicked spastic diplegia; and the fourth showed some similarity to torsion dystonia. Emotional or cognitive disturbance, or both, was seen in three. The correct diagnosis was suggested by fluctuating signs and symptoms, which worsened towards evening, but this was reached only after many years of handicap, hospital admissions, and invasive diagnostic procedures. Typically there was a prompt, pronounced, and sustained response to moderate doses of levodopa. Sleep recordings were obtained in three patients and showed increased body movements during rapid eye movement sleep. Several close relatives had periods of increased leg movements during sleep. It is suggested that hereditary dystonia responsive to levodopa should be considered as the diagnosis in children with fluctuating signs of motor disability syndromes, simulating torsion dystonia or spastic diplegia. Polysomnographic studies may be helpful in diagnosis and may also detect early or subclinical cases.

Child↗
PubMed1986

Neuromuscular involvement in glycogen storage disease type III.

Sixteen patients with glycogen storage disease type III (GSD III) aged 3 to 22 years underwent a detailed neuromuscular evaluation. A minimal impairment of skeletal muscle function was presented in eight patients, slight impairment in four and severe impairment in one patient. Serum creatinine phosphokinase (CPK) was elevated in all patients studied. In the nine patients, in whom electromyography (EMG) was performed; six exhibited a myopathic pattern while a "mixed" (neurogenic-myopathic) pattern was present in three. Muscle biopsies performed in 12 patients, revealed in all cases amylo-1,6,-glucosidase deficiency and biochemical as well as morphological evidence of glycogen accumulation. Two brothers suffered from late onset myopathy, which in the older sibling was associated with clinical, EMG and EM findings of a peripheral neuropathy. Fifteen patients had either electrocardiographic and or echographic evidence of cardiomyopathy. Observations based on this patient material suggest a widespread myopathy in GSD III patients with heterogeneous expression, while peripheral nerve involvement is rarely encountered.

Adolescent↗
PubMed1985

Pseudotumor cerebri in a boy with 11-beta-hydroxylase deficiency--a possible relation to rapid steroid withdrawal.

A boy who was known to suffer from adrenogenital syndrome due to 11-beta-hydroxylase deficiency was treated with appropriate steroid replacement, which fully compensated for the deficiency. An intercurrent febrile illness with anorexia and vomiting necessitated an abrupt discontinuation of steroids. He presented with classic signs of pseudotumor cerebri 3 days following steroid withdrawal. Our studies imply that a combination of steroid withdrawal and dehydration with increased sodium urinary excretion, caused rapid fluid shifts within the brain, resulting in intracranial hypertension. At present, when a considerable number of children are on long-term steroids for various reasons, pseudotumor cerebri should be considered in the list of complications during abrupt steroid withdrawal.

Adrenal Hyperplasia, Congenital↗