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Biomedical subjects

N Gadoth

Publications and source records attributed to N Gadoth.

At least 19 recordsLinked to original sources
PubMed1992

Dental and oral findings in patients with familial dysautonomia.

Familial dysautonomia is an inherited autosomal recessive disease found almost exclusively in Ashkenazi Jews. It is characterized by selective damage to the sensory, motor, and autonomic peripheral nervous system. The main clinical features include decreased pain sensation, impaired temperature and blood pressure regulation, lack of tearing, absent tendon reflexes, and fungiform papillae on the tongue. The purpose of this study was to explore in depth and to verify the oral and dental status in familial dysautonomia. Twenty-two patients and 44 match-paired healthy persons of Ashkenazi descent were examined. Patients and parents had only little concern for their oral condition. Caries prevalence was lower than normal and plaque accumulation increased in all patients. Dental trauma was found in 59% of the patients, and 32% showed orodental self-mutilation. Dental age was within normal range, and dental arch measurements implied proportionally small jaws and little crowding. The low caries rate may be related to the known "hypersalivation" in familial dysautonomia and/or a possible change in the salivary composition and content, caused by chronic autonomic denervation.

Adolescent↗
PubMed1991

The prognostic significance of glial fibrillary acidic protein staining in medulloblastoma.

Histologic and immunohistochemical properties of 53 medulloblastomas were analyzed with regard to clinical features and survival rate. No correlation was found between survival rate and histologic features of the tumor, such as desmoplastic reaction, number of mitoses, hemorrhages, necrosis, endothelial proliferation, glomerular arrangement, calcifications, rosettes, or oligodendroglial cells. However 82% of the patients with positive glial fibrillary acidic protein (GFAP) staining in numerous tumor cells survived more than 5 years; only 30% survived that long if their tumor cells were GFAP negative (P = 0.0093). This significant difference was not related to the mode of therapeutic protocol used. The authors suggest that GFAP staining may be a useful prognostic tool in medulloblastoma.

Adolescent↗
PubMed1991

The value of sleep recording in evaluating somnambulism in young adults.

Somnambulism (SOM) is a benign childhood sleep disorder which may persist until young adulthood. The diagnosis relies heavily on the history, and no polysomnographic (PSG) criteria have yet been defined. The present study attempts to evaluate the role of whole-night polysomnographic recording in the investigation of SOM. The PSG records of 24 sleepwalkers, 18-25 years old, and 12 age-matched controls, were analysed. Sleepwalkers had remarkably more epochs containing hypersynchronous delta waves (HSD) (59.6 +/- 60.1 vs. 1.7 +/- 3.2; P less than 0.0001), a higher proportion of HSD/total time spent in stage 3-4 (24.9 +/- 21.1% vs. 1.1 +/- 2.0%, P less than 0.0002), and more stage 3-4 sleep interruptions (8.4 +/- 5.7 vs. 3.7 +/- 1.7, P less than 0.004). They also tended to have a larger proportion of their sleep time in stage 3-4 (30.6 +/- 11.7% vs. 22.6 +/- 6.8%; P less than 0.07). Although their sensitivity and specificity have yet to be more fully investigated, these seem to be quantitative, easy-to-use variables which may characterize adult SOM and may aid in its proper diagnosis.

Adolescent↗
PubMed1991

Three modality evoked potentials in Charcot-Marie-Tooth disease (HMSN-1).

Sixteen patients with dominant hereditary motor-sensory neuropathy type I (HMSN I), members of 5 families, underwent trimodality evoked potential studies. All patients had clinically normal optic nerves. History of deafness was present in 3 patients and sensory-neural hearing defect was found in 5 of 7 patients in whom audiometry was obtained. In 43.7 percent of the subjects significant prolongation of P100 of the VEP was found. Prolongation of N19 of the SEP was found in all 12 subjects examined. Significant bilateral prolongation of peak I of the ABEP was found in 37.5 percent of the subjects and in 50 percent of the ears examined: these findings indicated that in addition to peripheral nerves, the myelin of the optic and cochlear nerves is also affected in HMSN type I.

Adolescent↗
PubMed1991

Short pancreas in polysplenia syndrome.

Two cases with the polysplenia syndrome also had a short pancreas. To the best of our knowledge the radiologic characteristics of this anatomic anomaly have not yet been reported. Both cases were asymptomatic with regard to their anomaly-complex. The radiologic findings of the short "pancreas" are similar to the only pathologic description of this association. The awareness of this association led to the diagnosis of the second case after a few months only. A physician performing ERCP in the polysplenia syndrome should keep in mind the possible presence of the "short pancreas" when the whole length of the pancreatic duct is not visualized.

Abnormalities, Multiple↗
PubMed1990

[Malignant angioendotheliomatosis].

Malignant angioendotheliomatosis is one of the terms used to describe the appearance of neoplastic cells resembling endothelium within the lumen of small and medium-sized blood vessels, mainly in the cerebral and meningeal vascular beds. A fatal systemic disease with dementia as the leading neurological manifestation may be associated with these pathological findings. Only recently has it become evident that this disease is a unique intravascular form of large cell lymphoma, affecting mainly the cerebral blood vessels but also present in the lumen of many small and medium-sized blood vessels in the systemic circulation.

Blood Vessels↗
PubMed1990

[The diagnostic value of CT and MRI studies in brain-stem tumors].

The combination of lower cranial nerve palsy and contralateral motor impairment, presenting subacutely and following a progressive course in a child or young adult, suggests the possibility of brain-stem tumor. However, if the CT scan is normal, the diagnosis of multiple sclerosis or a vasculitis is frequently considered. Since CT scans may miss lower brain-stem tumors, MRI is recommended as the definitive diagnostic test in such cases.

Brain Neoplasms↗
PubMed1989

Secondary amenorrhea in two sisters with hypogonadotropic hypogonadism and progressive cerebellar ataxia.

The association of familial hypogonadism with progressive cerebellar ataxia is only rarely encountered. Both primary hypergonadotropic and secondary hypogonadotropic hypogonadism may appear with cerebellar ataxia. However, many of these patients suffer from a variety of neurological and/or somatic malformations. Females, which are relatively rarely affected, display primary amenorrhea. In this report, two sisters presented with secondary amenorrhea prior to the appearance of progressive cerebellar ataxia and were found to have hypogonadotropic hypogonadism. This unique family displays clinical evidence for the presence of a possible common mechanism responsible for progressive hypothalamic and cerebellar impairment of late onset.

Adult↗
PubMed1989

The interferon system in subacute sclerosing panencephalitis and its response to isoprinosine.

Plasma interferon activity (IFN) and its spontaneous and stimulated production by peripheral blood mononuclear cells (PBMC) was studied in 11 patients with subacute sclerosing panencephalitis (SSPE) and age-matched healthy controls. The patients, similar to the healthy controls, had no detectable plasma IFN activity. However, their PBMC failed to produce IFN in response to stimulation with poly I:C and PHA. After isoprinosine administration to 7 patients for several days a significant increase in plasma IFN activity was observed and their PBMC responded to stimulation by producing IFN. The long-term effect of isoprinosine on the IFN response was evaluated in 3 patients who had been treated for 57-88 days. Induction of the abolished IFN production was observed with initiation of therapy. However, discontinuation of isoprinosine for 10 days resulted in recurrence of the inactivation state of the IFN system.

Adolescent↗
PubMed1989

Radiological colpocephaly: a congenital malformation or the result of intrauterine and perinatal brain damage.

The term colpocephaly, meaning disproportional enlargement of the occipital horns of the lateral ventricles, was considered in the past to be a distinct congenital malformation acquired in early intrauterine life. During the last few years several cases were reported in whom a variety of intrauterine and perinatal causes could be associated with this radiological picture. We report on 9 children with radiological colpocephaly in whom intrauterine and/or perinatal injury to the developing brain seemed to be the cause of colpocephaly. It is evident from our observations that "radiological colpocephaly" is a non-specific finding caused frequently by CNS damage acquired during intrauterine and perinatal life.

Brain Diseases↗
PubMed1989

The regional cerebral blood flow in familial dysautonomia.

Regional cerebral blood flow (rCBF) was measured during rest by the Xenon 133 inhalation method in 8 patients with familial dysautonomia (FD). The rCBF data were compared with those obtained in a group of 55 age-matched normal subjects. In 5 moderately affected patients there was a significant increase in the rCBF, while in one severely affected female, rCBF was markedly decreased. In two mildly affected patients, rCBF was normal and similar to controls. It is suggested that the increases in rCBF in FD may compensate for a yet undetermined autoregulatory failure of cerebral vasculature.

Adolescent↗