Hypertonic saline treatment in children with cerebral edema.

OBJECTIVE: To compare the efficacy and side effects of hypertronic saline and mannitol use in cerebral edema. DESIGN: Retrospective study. SETTING: Pediatric intensive care unit. SUBJECTS: 67 patients with cerebral edema. METHODS: Patients with cerebral edema treated with either mannitol or hypertronic saline (HS) (Group II: n = 25), and both mannitol and HS (Group III: n = 20) were evaluated retrospectively. Cerebral edema and increased intracranial pressure were based on the clinical and/or radiological (CT, MR) findings. When treating with both mannitol and HS (Group IIIA), if patients serum osmality was greater than 325 mosmol/L, mannitol was stopped and patients were treated with only HS (Group IIIB). All patients were closely monitored for fever, pulse, blood pressure, central venous pressure (CVP), oxygen saturation, volume of fluid intake and urine output. Mannitol was given at a dose of 0.25-0.5 g/kg while the hypertonic saline was given as 3% saline to maintain the serum-Na within the range of 155-165 mEq/L. RESULTS: There was no statistically significant difference in terms of Glasgow coma scale, age, gender, and etiologic distribution between the groups. And also distribution of the other treatments given for cerebral edema is not significiant. Mannitol was given for a total dose of 9.3 +/-5.0 (2-16) doses in Group I, and 6.5 +/-2.8 (2-10) doses in Group III. Hypertonic saline was infused for 4-25 times in Group II. Although there was no statistically significant difference in the highest serum Na and osmolarity levels of the groups, duration of comatose state and mortality rate were significantly lower in Group II and Group III A B. Patients who received only HS were subdivided according to their serum Na concentrations into 2 groups as those between 150-160 mEqL and those between 160-170 mEqL. The duration of comatose state and mortality was not different in patients with serum-Na of 150-160 mEqL and in patients with 160-170 mEqL in the hypertonic saline receiving patients. Four patients in the group II developed hyperchloremic metabolic acidosis and 2 patients in the group I had hypotension. As two patients in group II had diabetes insipidus and one patient had renal failure in group I, the treatment was terminated. The causes of death were septic shock, ventilator associated pneumonia with acute respiratory distress syndrome, progressive cerebral edema and cerebral edema with pulmonary edema. Multivariate analysis showed that age, gender, cause of cerebral edema, electrolyte imbalance, hyperglycemia and hyper-ventilation had no significant impact on outcome. CONCLUSION: Hypertonic saline seems to be more effective than mannitol in the cerebral edema.

Giant axonal neuropathy in two siblings: clinical histopathological findings.

OBJECTIVE: Giant axonal neuropathy is a rare, severe autosomal-recessive neurologic disease affecting both the peripheral and the central nervous system. In this article, we describe a detailed clinicopathological report of two affected sibs from a consanguineous Turkish family. PATIENTS: The index patient was a 6.5-year-old girl. Her intellectual development was normal. At the age of 3, her parents noticed progressive lack of balance and deterioration of motor skills. On examination, she had paresis and sensory loss more marked distally. Her mental status was normal. Her older brother had similar findings. RESULTS: Electrophysiological studies of young patients showed decreased median and ulnar nerve conduction velocities, absent peroneal motor potential, absent sensory nerve potentials and an EMG suggesting a neurogenic pattern. MRI showed mild cerebral and cerebellar atrophy. The nerve biopsy showed moderate myelinated nerve fibres loss, several regenerative clusters and multiple giant axons. Focal demyelination, hypertrophic "onion pulp" changes and endoneural fibrosis were also seen. Immunohistochemically, neurofilament protein accumulation was detectable in giant axons. CONCLUSIONS: This consanguineous family with two affected siblings and healthy parents complies with autosomal-recessive inheritance in GAN. In the majority of reported GAN cases, CNS involvement is described early in the course of the disease, but these patients did not present any sign of CNS involvement. GAN is a rare genetic disease of childhood involving the central and peripheral nervous systems. The diagnosis is easy with clinical, electrophysiological, and histopathological features, if it has been done. Early diagnosis is important, because of possible prenatal diagnosis.

Changing epidemiological features of subacute sclerosing panencephalitis.

BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a chronic central nervous (CNS) system infection caused by measles virus. Because changing immunization practices affect the epidemiology of measles and consequently SSPE, we examined the epidemiological data of our SSPE registry. MATERIALS AND METHODS: Age of onset, age at onset of measles, duration of Latent period and immunization status were examined in cases recorded at the SSPE Registry Center in Turkey between 1975 and 1999. RESULTS: Age of onset diminished from 13 years before 1994 to 7.6 years after 1995; age at onset of measles declined from 29 months to 20 months and the Latent interval from 9.9 years to 5.9 years. Age at onset of measles and immunization status did not directly affect the duration of the Latent period. CONCLUSION: Although its incidence has decreased in Turkey, SSPE has been seen at younger ages in recent years. This change cannot be attributed solely to younger age at onset of measles. Factors affecting the duration of the Latent period should be investigated further.

Renal tubular dysfunction in epileptic children on valproic acid therapy.

To investigate the effects of valproic acid (VPA) on renal tubular function, we examined 15 ambulatory children with epilepsy who received VPA for at least 6 months. None of the patients had mental retardation. Fourteen age- and sex-matched children were used as a control group. No statistically significant differences were found between patients and control subjects with respect to blood urea nitrogen (BUN), creatinine (Cr), uric acid, creatinine clearance (Ccr), tubular reabsorption of phosphorus (TRP), urinary Ca:creatinine ratio, urinary pH and mean urinary beta2-microglobulin concentrations (P>0.05). Protein and glucose in patient urine samples were negative. Urine microscopic examinations and amino acid chromatographies of patients were also normal. However, significant differences were found between patient and control groups with respect to mean urinary N-acetyl-beta-D-glucosamine:creatinine ratio (NAG:Cr) and mean urinary malondialdehyde:creatinine (MDA:Cr) ratio (P<0.05). In conclusion, ambulatory children with epilepsy taking VPA therapy may develop proximal renal tubular dysfunction. Although this finding is clini-cally insignificant, it should be kept in mind during VPA therapy.

Relapse risk analysis after drug withdrawal in epileptic children with uncomplicated seizures.

In an attempt to find the risk of relapse and factors predictive of risk of relapse, 97 children with epilepsy, withdrawn from their medication, followed in our outpatient clinic from 1990 to 1995 were included in this study. The overall relapse rate was 20.6%. All relapses occurred within 2 years after withdrawal started. Female gender, age at onset of seizures of more than 2 years, and the duration of withdrawal were found to be significant risk factors in relapse rate following univariate analysis. However, gender was not found to be significant in multivariate analysis. All other factors, including the duration of seizures prior to starting antiepileptic drug (AED) treatment, the number of seizure before the start of AED treatment, the period between AED induction and control of seizures, diagnostic yield from electroencephalogy (EEG) at diagnosis, the number of seizures after the onset of AED therapy, length of seizure-free period, aetiology of the seizures, a history of epilepsy in the immediate family, or previously experienced febrile convulsions were not significant factors in relapse rate. Significant risk factors for relapse rate also significantly affected relapse time. We conclude that when AED therapy is withdrawn from children with uncomplicated epilepsy, as in our patients, the two important risk factors, age at onset of seizures and the duration of withdrawal, can predict a poor prognosis with a higher relapse rate: this needs taking into consideration.

Kearns-Sayre syndrome. A case report.

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder. There is a large-scale mitochondrial DNA (mtDNA) deletion in most of the case. In this article, a case of KSS who has progressive external ophthalmoplegia (PEO), retinitis pigmentosa (RP), complete heart block, encephalopathy attacks, type-1 diabetes mellitus, ragged-red fiber (RRF) and lactic acidosis is presented and discussed in light of the literature available on this subjects. Diagnosis is confirmed by determination of mtDNA deletion.

Serum and hair zinc levels in epileptic children taking valproic acid.

This study was performed to investigate the serum and hair zinc levels in patients epilepsy diagnoses who were intended to be put on valproic acid (VA) monotherapy and had never ingested antiepileptics before. A total of 16 patients having normal growth, development and nutrition was selected as Group 1, and Group 2 was made up of 10 patients who had received VA monotherapy for 2 yrs or more and had normal growth, development, and nutrition characteristics. A control group (Group 3) was formed of 15 subjects who applied to the hospital for upper respiratory tract disorders. Serum and hair samples were taken for zinc assays from the Group 1 patients on the d 0, 15, 30, 45, 60, 75, and 180. Groups 2 and 3 were sampled only once, and zinc levels were determined. We found that both serum and hair zinc levels in Group 1 were higher than those of Group 2 and control group before the beginning of VA therapy, but they returned to normal during VA treatment. There was no zinc deficiency, and zinc replacement treatment may therefore be considered as unnecessary.

Startle disease--hyperekplexia (two sibling cases).

Two sisters aged 12 and 13 applied to our hospital with complaints of jerking tonic contraction triggered by sudden noise. Diffuse bilaterally polyspike-wave discharges appeared on waking electroencephalography and during that time a startle-induced tonic contraction was seen. Their neurological examinations and neuro-radiological imaging were normal except for hyperreflexia. We presented these cases because of being rare.

Intracerebral tuberculoma in children with tuberculous meningitis.

Not all patients with tuberculosis develop tuberculoma during the disease or antituberculous therapy. Therefore, we compared various parameters in patients with and without tuberculoma and presented the results. We formed two groups from the patients with tuberculous meningitis: Group I consisted of 18 patients with tuberculoma, and Group II consisted of 18 randomly selected patients without tuberculoma. Significant difference between the groups was found with respect to level of consciousness and CSF-glucose level (p < 0.05). The consciousness level was more depressed and the CSF-glucose level more decreased in the group without tuberculoma. These findings were discussed.

Primary rhabdomyosarcoma of the heart presenting with increased intracranial pressure.

An 18-month-old boy presented with signs of increased intracranial pressure. Computed tomographic scans of the brain and lungs showed multiple masses in the brain and lungs. Echocardiography revealed homogeneous, irregular pericardial thickening, minimal pericardial effusion, and irregular echogenic areas in the left ventricular inferoposterior and apical regions. Postmortem examination of tumor tissue showed that the masses were primary rhabdomyosarcoma of the left ventricle with multiple metastases. Primary rhabdomyosarcoma of the heart is uncommon, especially in patients under 2 years of age.

Suprasellar tuberculoma causing endocrinologic disorders and imitating craniopharyngioma.

A patient is reported with a suprasellar tuberculoma which imitated a craniopharyngioma on computed tomography scan and magnetic resonance imaging of the brain. This lesion developed during irregularly used antituberculous therapy and caused endocrinologic disorders 3 years after the original diagnosis of tuberculous meningitis. Surgery was performed to confirm the diagnosis and also to decompress the optic pathways. Five months later, the lesion disappeared completely after appropriate antituberculous treatment.

Tuberculous meningitis in children.

Between May 1988 and November 1992 the data from 52 patients with tuberculous meningitis (TBM) were noted down for their symptoms and signs, BCG vaccines, PPD tests; clinical, laboratory, radiologic and microbiologic findings. These data were discussed by means of literature knowledge. Cranial computed tomography (CT) demonstrated hydrocephalus (HC) in 98% of the patients. There was a statistically significant difference among the clinical stages on admission in respect to prognosis (P < 0.05). In addition, there was also a significant relationship between prognosis and HC (P < 0.05). However, we did not find any significant relationship between parenchymal involvement, basilar meningitis and prognosis (P > 0.05).