Acute flaccid neonatal paraplegia: a case report.

A 3-day-old neonate became acutely and irreversibly paraplegic below L1/L2 after umbilical artery catheterization. The paraplegia was attributed to infarction of the spinal cord because of thrombosis of the artery of Adamkievicz or injection of drugs through the catheter into the spinal cord circulation. Catheterization of a more peripheral artery or placement of the umbilical catheter tip at a lower level in the aorta may prevent similar complications.

High fever after flexible bronchoscopy and bronchoalveolar lavage in noncritically ill immunocompetent children.

Flexible bronchoscopy (FB) and bronchoalveolar lavage (BAL) have been applied increasingly to the evaluation of pulmonary disease in children. Although several complications have been reported following FB and BAL, high fever after BAL in immunocompetent children has not previously been reported. To determine the frequency, clinical characteristics, and outcome of these complications in children who developed high fever post-BAL, we retrospectively reviewed all bronchoscopic procedures done on an outpatient basis between August 1995 and July 1997. We identified 78 immunocompetent noncritically ill children who had undergone FB and BAL as an outpatient procedure for evaluation of underlying pulmonary disease, of whom 13 (17%) developed temperature (T) higher than or equal to 39 degrees C (fever group). The 13 patients in the fever group had a median age of 10 (range, 4-48) months and a reported T of 39.4 degrees C (39.1-40.6 degrees C) occurring 7.5 (4-12) hr after BAL. To determine if there were differences in clinical or BAL fluid (BALF) characteristics, we compared each child in the fever group to two children in the nonfever group, based upon primary indications and age. There were no differences in demographic or clinical characteristics between the two groups. Lymphocyte concentrations in BALF were significantly reduced in the fever group (P = 0.03). An abnormal BALF cell differential (defined as one or more of the following: neutrophils >10%, lymphocytes >30%, or eosinophils >1%) was significantly more common in the fever group (P = 0.008, odds ratio 3.6). We conclude that high fever is a frequent adverse event following BAL in noncritically ill immunocompetent children with underlying pulmonary disease. Pre-BAL clinical characteristics are not associated with development of high fever. However, the finding of an abnormal BALF cell differential is strongly associated with development of high fever post-BAL.

Propanolol treatment of infantile polymyoclonia.

Two infants with the classical involuntary movements of polymyoclonia and opsoclonus were treated with propanolol in a dosage of 2 mg/kg/24 hr. There was a marked improvement in the abnormal movements in both children. No side effects of treatment were encountered.

Biphasic effects of imipramine in experimental models of epilepsy.

In a variety of laboratory models of experimental epilepsy, imipramine exerts a biphasic action on the CNS as manifested by antiepileptic properties at low doses and convulsant effects at higher doses. In mice, imipramine (17.5-25 mg/kg, i.p.) blocks maximal electroshock seizures while exerting little or no effect on pentylenetetrazol-induced seizures. In cats, imipramine (2.5-15 mg/kg, i.v.) reduces penicillin and estrogen-induced epileptiform discharge, shortens afterdischarge duration and elevates afterdischarge threshold. Higher doses in mice induce neurotoxicity, including clonic seizures. In cats, doses above 20 mg/kg intensify chemically and electrically induced seizures and induce spontaneous epileptiform episodes. Such a biphasic action of imipramine may limit the drug's clinical utility as an antiepileptic agent and may provide an interesting tool for studies of catecholamines and brain excitability.

The effects of antiepileptic drugs on estrogen-induced electrographic spike-wave discharge.

In locally anesthetized, paralyzed cats with bilateral conjugated estrogen (CE)-induced foci in sensory motor cortex, electrographic activity was characterized by 2 to 3 Hz spike and slow wave discharge. Commonly used anti-petit mal drugs (esthosuximide, trimethadione, acetazolamide and diazepam) all reduced CE-induced spike wave activity while diphenylhydantoin converted such activity into 9 to 12 Hz polyspike bursts separated by periods of interictal silence. Correlation appears to exist, therefore, between the ability of the drug to reduce CE-induced spike wave activity and its clinical utility in petit mal epilepsy. In addition to the above compounds, five drugs of less proven utility were evaluated. Of these, two benzodiazepine derivatives (clonazepam and clorazepate) were found to exert a potent and prolonged depressant action on CE-induced activity. The relation of CE to clinical petit mal epilepsy and the potential usefulness of CE as a laboratory model for the evaluation of anti-petit mal drugs are discussed.

The diaphragma sellae: its relationship to normal sellar variations in frontal radiographic projections.

Recognition of prolactin-secreting microadenomas has led to renewed interest in normal variations in sellar contour that may mimic pathologic changes. A study of sphenoid bones obtained at autopsy from 100 patients who had histologically normal pituitary glands was done to determine the relationship between the diaphragma sellae and variations in the floor of the sella turcica, as seen in frontal radiographs. Nineteen of 46 (41%) sphenoid bones with a complete diaphragm had a floor that was convex downward with a depth range of 1-2 mm. Thirty-one of 54 (57%) sphenoid bones with a defective diaphragm had a floor that was convex downward with a depth range of 0.5-6 mm. A positive correlation existed between the presence of a defect in the diaphragma sellae and the depth of the central depression of the floor, and also between the depth of any resulting intrasellar cistern and the depth of the central depression. Eight bones with large defects in the diaphragma sellae showed great convex downward bowing of the floor with a central depression of greater than 2 mm in each case. Despite a histologically normal pituitary gland, a defect in the diaphragma sellae may lead to expansion of the bony contour of the sella turcica.

Communicating hydrocephalus in children with genetic inborn errors of metabolism.

The authors have seen eight cases of communicating hydrocephalus in children with genetic metabolic disorders, namely, one mucopolysaccharidosis I (MPS I or Hurler syndrome), one MPS II (Hunter's disease), four MPS III (Sanfilippo syndrome) two of which were siblings, and two achondroplasias. The authors recommend surgical treatment of the latter but are doubtful about the former in which case hydrocephalus is only a contributing cause to severe dementia. In MPS hydrocephalus is due to accumulation of storage material in the piaarachnoid, as indicated by RISA cisternography carried out in six cases.

Immunological status in tuberous sclerosis.

Cellular and humoral immunity, and lymphoid organ pathology, have been investigated in 10 institutionalized patients with tuberous sclerosis and 10 institutionalized matched controls without the disease. Type and incidence of infections and tumours were reviewed for each group, as was current medication. Elevated serum IgM levels were found in the patients with tuberous sclerosis, but no immunological deficiency of either cellular of humoral immunity was found, nor was there a difference in infection between the groups. Only patients with tuberous sclerosis had evidence of neoplasia. No morphological or histological abnormalities of lymph nodes, spleen or thymus were present. Explanations for the difference between tuberous sclerosis and ataxia telangiectasia are discussed, together with the effect of immuno-surveillance on the development of malignancy.

The diaphragma sellae: its relationship to the configuration of the pituitary gland.

One hundred sphenoid bones that were removed at autopsy from patients without apparent pituitary disease and with histologically normal pituitary glands were examined. In 46 specimens the diaphragma sellae was complete. The majority showed convex downward bowing below the diaphragmatic line in both the lateral and frontal planes while the remainder were straight, corresponding to the diaphragmatic line. In only one specimen was the diaphragma sellae convex upward in both planes. These findings suggest that upward displacement of the diaphragma sella as seen of coronal and sagittal computed tomography (CT) may provide early evidence of an expanding pituitary lesion. Twenty of 54 defective specimens had a defect that was larger than 80% of the length of the diaphragma sellae. There was a correlation between the size of the defect and the depth of the intrasellar cistern that resulted from the downward extension of the suprasellar cistern. Such variations may lead to confusion in CT interpretation. The pituitary infundibulum was at or behind the level of the midpoint of the diaphragma sellae in all specimens. In the presence of a defect, the infundibulum usually retained its relationship to the posterior rim of the defect, and therefore when the defect was large the infundibulum was found adjacent to the dorsum sellae.