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Biomedical subjects

N Gadoth

Publications and source records attributed to N Gadoth.

At least 145 records · Page 8Linked to original sources
PubMed1982

Presence of fungiform papillae in classic dysautonomia.

Lack of fungiform papillae over the surface of the tongue is considered essential for the diagnosis of familial dysautonomia. We describe two cases with dysautonomia in which the diagnosis was delayed because numerous fungiform papillae were easily seen. Only a close inspection of the tongue with an ophthalmologic slit lamp revealed that each papilla was degenerated and atrophic. Lacking a clinical sine qua non or easily available diagnostic laboratory tests, fungiform papillae should be studied in detail to establish not only that their number is sufficient but also that their shape and structure are normal. The simple technique described enables confirmation of the diagnosis in children and newborns in whom familial dysautonomia is suspected.

Diagnostic Errors↗
PubMed1981

Unilateral pupillary dilatation during focal seizures.

Pupillary dilatation was observed in a young boy who had a sudden onset of right focal seizures. Clinical and laboratory investigation revealed the presence of a benign left frontal epileptic focus. Lacking signs of third cranial nerve compression, this transient pupillary abnormality could be caused by the contralateral frontal epileptic focus. Such a focus might inhibit dilatation of the homolateral pupil, thus permitting dilatation of the contralateral pupil only. Unilateral mydriasis as described hereby could also represent a pupillary "Todd's Paralysis" caused by the contralateral frontal epileptic focus.

Adolescent↗
PubMed1981

Further observations on sleep abnormalities in Kleine-Levin syndrome: abnormal breathing pattern during sleep.

In two adolescent and two adult patients with Kleine-Levin syndrome, polygraphic sleep recording performed during somnolent and non-somnolent periods revealed various forms of abnormal breathing patterns during sleep. These included periodic breathing and hypopnoeic episodes associated with brief arousals and, in one adult patient, a full blown sleep apnoea syndrome. It is suggested that abnormal breathing in sleep in this syndrome may result from central hypoexcitability.

Adolescent↗
PubMed1981

Permanent tetraplegia as a consequence of tetanus neonatorum. Evidence for widespread lower motor neuron damage.

It is generally believed that no permanent neurological damage is found among survivors of tetanus neonatorum. Newborns dying shortly after the onset of tetanus also lack significant neurological abnormalities. In adults a variety of neuromuscular lesions have been reported; however, a uniform pathological picture is absent. We report a case of a newborn with severe tetanus in whom striking evidence of anterior horn neuronal damage was documented, causing permanent nonprogressive tetraplegia. We suggest that the mechanism responsible for this lesion involves the retrograde axoplasmic flow of tetanus toxin reaching the spinal cord via nerve endings in the infected umbilical cord stump.

Atrophy↗
PubMed1981

Hereditary heat-labile hexosaminidase B: its implication for recognizing Tay-Sachs genotypes.

Two pairs of alleles, at the two loci of hexosaminidase (HEX), were found to segregate in an Arab inbred family: the normal and the mutant Tay-Sachs (TSD) alleles of HEX A, and the normal and a mutant allele of HEX B. Since the mutant HEX B is heat labile, no reliable identification of TSD genotypes can be obtained in its presence, as long as the proportions of HEX A and B are estimated by the routinely used heat-inactivation method. The genotypes may be correctly identified in such cases by separation of the two isoenzymes on ion-exchange chromatography, estimating their individual activities, and calculating the ratio between them. Of the nine genotype combinations possible with these two pairs of alleles, five have been identified in the reported family by this procedure.

Adult↗
PubMed1980

Posterior fossa subdural hematoma in a normally delivered, full-term newborn.

A large subdural hematoma of the posterior fossa was diagnosed by computerized tomography in a normally delivered, full-term newborn. This potentially treatable condition has been previously diagnosed in only 13 reported cases during the last 40 years. It is conceivable that the routine use of CT scan in newborns who show progressive neurological dysfunction will facilitate the rapid detection and treatment of similar cases.

Brain↗
PubMed1980

Primary and acquired forms of moyamoya syndrome. A review and three case reports.

Moyamoya is the name given to an angiographic picture consisting of bilateral intracranial carotid artery occlusion associated with telangiectatic vessels in the region of the basal ganglia. It appears that patients with this angiographic finding can be divided into two distinct clinical groups. The first consists of children with a "primary" form of moyamoya that causes alternating hemiplegia, frequently of nonprogressive nature. The second consists of children and adults with various underlying diseases who develop an "acquired," usually progressive form of moyamoya. The striking predominance of Japanese patients with primary moyamoya and its high familial occurrence suggests that the primary form represents a hereditary malformation of the cerebral vascular bed. We report three additional cases of moyamoya. One case represents the primary form and the two are examples of the acquired form in a child and an adult.

Arterial Occlusive Diseases↗
PubMed1979

Acute anterior myelitis complicating West Nile fever.

A healthy young man developed acute anterior myelitis resembling the "polio syndrome." He had visited an area (the Gulf of Suez) in which West Nile fever is endemic prior to his illness. The course of his febrile illness, the spinal fluid findings, and complement fixation antibody titers established the diagnosis of West Nile fever. Meningoencephalitis is a serious, and previously the only known, complication of this usually benign febrile illness. This is the first report, to our knowledge, of another neurological complication, namely acute anterior myelitis.

Acute Disease↗
PubMed1979

Rheumatoid arthritis during the first year of life. A case report and review of the literature.

A 20-week-old female, first examined at 14 weeks of age for daily "spiking" fever and skin rash, subsequently developed signs of rheumatoid arthritis. The onset of rheumatoid arthritis during the first weeks of life is rare and its diagnosis may be difficult. It seems that onset during the later months of the first year is more frequently encountered. A review of the relevant publications in English disclosed that onset during first year was noticed in 1.02 percent of the patients, while in a study from Germany 6 to 9.9 percent of cases started during their first year.

Age Factors↗
PubMed1979

Sleep patterns in Kleine-Levin syndrome.

Diurnal and nocturnal sleep records were obtained from a male and a female with Kleine-Levin syndrome, during excessive daytime sleep attacks and while they were asymptomatic. A common pattern of abnormal sleep was seen in both patients even during the asymptomatic period. The female, aflicted with a severe form of periodic hypersomnia, showed nocturnal and diurnal sleep onset REM periods. The different pattern of sleep abnormality in the female could be an expression of the severity of her symptoms or might indicate a variant of sleep abnormality present only in females with Kleine-Levin syndrome.

Adolescent↗
PubMed1979

Congenital vascular malformation of spinal cord simulating diastematomyelia.

Diastematomyelia is usually caused by a midline bony, cartilaginous or fibrous spur. A case is presented with splitting of the spinal cord by a large arteriovenous malformation at the thoracolumbar junction. A hairy skin patch, kyphoscoliosis, vertebral anomalies and an arachnoidal cyst were found adjacent to the vascular and neural malformations.

Arteries↗
PubMed1979

Cat-scratch disease presenting as status epilepticus. A case report.

A 12-year-old Bedouin boy presented with sudden asymmetrical status epilepticus. The presence of a scalp wound, rubbery cervical lymphadenopathy and a history of contact with cats suggested the diagnosis of cat-scratch disease complicated by encephalopathy. The diagnosis was supported by histological examination of a cervical lymph node and a positive skin test with cat-scratch antigen. The rapid establishment of the diagnosis avoided the necessity of elaborate and complicated neuroradiological investigations.

Cat-Scratch Disease↗