On the problem of essential and secondary moyamoya and vascular dysplasia in Down syndrome.
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Blood Vessels↗
Biomedical subjects
N Gadoth
Publications and source records attributed to N Gadoth.
Clozapine for tardive dyskinesia in adolescents.
Thousands of adolescents use neuroleptic drugs over extended periods of time for a wide range of mental disorders. One of the most severe adverse effects of neuroleptic drugs is tardive dyskinesia (TD), for which no successful treatment is currently available. Clozapine is a known atypical neuroleptic drug with few extrapyramidal side effects. It has been suggested that clozapine may be beneficial for the treatment of tardive dyskinesia in adolescents. However, the efficacy of the drug in adolescents is unknown. This report describes the beneficial effect of clozapine on tardive dyskinesia in two adolescents with schizophrenia.
Adolescent↗
Paraneoplastic cerebellar degeneration heralding fallopian tube adenocarcinoma.
The objective of this paper is to describe an 81-year-old woman with subacute cerebellar degeneration due to fallopian tube adenocarcinoma. Serum anti-Yo antibodies were used to screen for pelvic malignancy. Their presence led to a meticulous search, which included bilateral salpingoophorectomy. Subsequently an occult fallopian tube adenocarcinoma was discovered. This case report highlights the diagnostic value of antineuroneal antibodies in females with subacute neurologic impairment in the form of paraneoplastic syndrome.
Adenocarcinoma↗
Oro-dental management of patients with familial dysautonomia.
Oro-dental abnormalities are quite frequent in familial dysautonomia. The practitioner should be familiar with this disorder, which represents a "human model" for chronic progressive oro-dental denervation. Self-injurious behavior is caused mainly by profound sensory loss. An appropriate preventive and therapeutic approach that was developed in our clinic and adapted for the special hazards of dentistry in youngsters with systemic autonomic dysfunction may improve the life quality of this particular population.
Adolescent↗
Intravascular malignant lymphomatosis: a cause of subacute dementia.
Intravascular malignant lymphomatosis (IML) is a rare disease characterized by proliferation of neoplastic cells of lymphoid origin within small blood vessels. The median survival of IML patients is only 6 months. Any organ can be affected, with or without clinical expression. Although skin lesions are classic, they are relatively uncommon (28%). Neurological symptomatology (which evolves over a few weeks) is the most common clinical expression (83%). Dementia is the most common neurological symptom that occurs in about half of the patients with central nervous system pathology, and is associated with poorer prognosis. The diagnosis is confirmed by histology but, except for lung, biopsies are not sensitive and are helpful only when performed in the symptomatic organs; furthermore, when associated with anesthesia, they can be followed by dramatic worsening of the patient's condition. Elevated LDH is a good indicator of IML in patients with subacute neurological symptomatology, especially if associated with signs suggestive of other organ involvement.
Brain Neoplasms↗
Mobius syndrome and Poland anomaly: case report and review of the literature.
A 6-year-old boy had the combination of Mobius Syndrome and the Poland anomaly. Although rare, this association is repeatedly encountered. The absence of the pectoral muscle in the Poland anomaly supports the theory that in both conditions the muscular and skeletal abnormalities are due to a common fetal mesodermal defect.
Child↗
Cyclic esotropia with minimal brain dysfunction.
An eight year old with a long history of hyperkinesis and learning difficulties compatible with the diagnosis of minimal brain dysfunction developed alternate day squint known as Cyclic Esotropia. No other abnormal cyclic phenomena such as alterations in sleep structure, diurnal variations in hemoglobin, hematocrit, electrolytes, body temperature, blood pressure or pulse rate was found. In view of the fact that cyclic esotropia may represent an impairment of the "biological clock" thought to be located in the hypothalamus, detailed studies of the hypothalamic hypophyseal axis were performed and found to be normal. It is suggested that if such a biological clock is present, its location is remote from the neural circuits involved in hypothalamic-hypophyseal hormonal release.
Attention Deficit Disorder with Hyperactivity↗
Can salivary composition and high flow rate explain the low caries rate in children with familial dysautonomia?
PURPOSE: Extremely low caries rate and increased major salivary gland flow rare have been previously reported in children with familial dysautonomia (FD). The purpose of this study was to explore the possibility that, in addition to increased salivary flow, children with FD have alterations in their salivary components, which may suggest an explanauon to their low caries rate. METHODS: Whole unstimulated and stimulated saliva samples were collected from 13 children with FD who were found to be caries free, and from 28 age- and ethnic-matched healthy children, 15 caries-free children and 13 caries-affected children. The electrolyte and protein content of the unstimulated saliva and the microbial count and buffering capacity of the stimulated saliva were determined. RESULTS: Children with FD had the highest salivary flow rate and the lowest levels of mutans streptococci and lactobacilli, as well as the lowest concentration of chloride, magnesium, total protein and IgA. Healthy caries-affected children displayed the highest mutans streptococci and lactobacilli levels and lysozyme concentration, concomitantly with the lowest potassium and calcium concentrations. CONCLUSIONS: The results of this investigation suggest that the caries-free state in FD may be associated with high salivary flow rate, while in healthy children, low caries rate may be associated with high salivary calcium concentration.
Adolescent↗
Transient regional and migratory osteoporosis. A possible neural mechanism.
Transient osteoporosis of the hip is a rapidly developing painful osteoporosis of benign nature and as yet unexplained pathophysiology. We add 5 new cases of transient osteoporosis, 4 of which are regional and 1 migratory, to 70 cases abstracted from the literature. Our review of the literature confirmed that there are two major subgroups of transient osteoporosis: regional and migratory. Two of our cases exhibited abnormal neurological findings. A possible neural mechanism related to regional osteoporosis should be further explored by a meticulous neurological and electrophysiological workup of such patients.
Adult↗
Sciatic pain: a diagnostic pitfall.
Neurological signs such as lower spinal cord lesions and/or femoral neuritis may be the presenting symptoms of a dissecting abdominal aortic aneurysm (AAA). We describe a patient in whom a severe sciatic pain was the presenting symptom of a non-dissecting AAA. The resection of the vascular mass resulted in dramatic relief of sciatic pain, thus indicating that the neurological impairment was caused by the non-dissecting aneurysm.
Aorta, Abdominal↗
I.v. amantidine sulfate for extrapyramidal crisis.
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Amantadine↗
Osteoid osteoma of the proximal femur simulating spinal root compression.
In a 6 1/2-year-old boy and a 9 1/2-year-old girl, spinal root compression was suspected on the basis of muscle wasting and weakness, diminished deep tendon reflexes, and severe pain, all in a radicular distribution. However, extensive and invasive neuroradiologic evaluation failed to demonstrate compression of spinal roots. Only several months later was the correct diagnosis of osteoid osteoma reached. In one patient, the tumor was removed and on histologic examination groups of unmyelinated nerve fibers were found near the center of the tumor nidus, generally surrounding small arterioles. In the other patient parental consent was not given for the operation. The symptoms gradually subsided, although the typical radiologic features of the tumor went unchanged. The presence of unmyelinated autonomic nerve fibers in the nidus of osteoid osteoma may explain the severe pain commonly experienced by the patients. The spinal origin of such fibres can account for the radicular syndrome, while the localization of pain and sensory findings remote from the tumor site might be explained by a mechanism similar to that responsible for "referred pain."
Child↗
The vestibulo-ocular reflex and seasickness susceptibility.
VOR parameters were compared in subjects at the extremes of the seasickness susceptibility scale. Thirty-nine subjects highly susceptible to seasickness and 30 nonsusceptible subjects participated in the study. The VOR was evaluated by the Sinusoidal Harmonic Acceleration (SHA) test at frequencies of 0.01, 0.02, 0.04, 0.08, and 0.16 Hz. In subjects susceptible to seasickness, VOR gain was significantly higher at 0.02 and 0.04 Hz, and phase lead was significantly lower at 0.01, 0.02, 0.04, and 0.08 Hz, than in nonsusceptible subjects. Our findings are in agreement with the notion that the vestibular response will be more intense in subjects susceptible to motion sickness. The present results support the contention that a natural insusceptibility, or increased resistance to seasickness produced by adaptive responses to repeated sea exposures, may be reflected by lower VOR gain and higher phase lead.
Acceleration↗