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Biomedical subjects

A Shuper

Publications and source records attributed to A Shuper.

At least 37 records · Page 2Linked to original sources
PubMed1997

Cure of recurrent medulloblastoma: the contribution of surgical resection at relapse.

BACKGROUND: Recurrent medulloblastoma has long been considered universally fatal. In spite of attempts to improve its treatment, only rarely have long term survivors been documented in the world's medical literature. Although the treatment of primary medulloblastoma is well established and includes surgical excision, postsurgical irradiation, and, more recently, chemotherapy, there is no established treatment for its recurrence. Current guidelines dictate the experimental use of chemotherapy, and there have been some encouraging results; yet no increase in long term survival has been documented. METHODS: This article presents a case of cured recurrent medulloblastoma in which the only intervention at the time of recurrence was surgical excision. In addition, a review of the literature is presented, which evaluates chemotherapeutic trials involving cases of recurrent medulloblastoma. RESULTS: The authors were surprised to find that the longest survivors of multiple trials had undergone reoperation at the time of recurrence. The authors' case, as well as cases described in the literature, point to a valid treatment alternative largely ignored in the management of recurrent medulloblastoma. CONCLUSIONS: Reoperation of recurrent brain tumors has received surprisingly little attention in the medical literature. Yet, as this case and a review of the literature demonstrate, this is a useful intervention.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1997

Optic chiasm glioma, electrolyte abnormalities, nonobstructive hydrocephalus and ascites.

A 4-year-old girl with optic chiasm glioma (OCG), nonobstructive hydrocephalus and ventriculoperitoneal shunt is described, in whom marked ascites developed. The ascitic fluid was protein-rich and its amount correlated with cerebrospinal fluid (CSF) protein. The CSF protein level and the amount of ascitic fluid were influenced by chemotherapy. Very unusual hypernatremia, up to 190 mEq/l with no associated alteration in mental status, was also found. It is suggested that altered absorption ability owing to the high protein content was the cause of both the nonobstructive hydrocephalus and the ascites. The unusual well being with very high sodium concentrations may have resulted from osmoreceptor dysfunction, presumably caused by hypothalamic involvement as well as by the high CSF protein. This combination of findings may point toward specific characteristics of OCG. In an effort to reduce the amount of the ascitic fluid, a further chemotherapeutic trial may be done, before converting the shunt to the vetriculoatrial system.

Ascites↗
PubMed1997

Ultrasonography of the optic nerves: clinical application in children with pseudotumor cerebri.

OBJECTIVE: Pseudotumor cerebri (PTC) in children has a wide spectrum of clinical presentations, from headache, which may be posterior and associated with nausea, vomiting or diplopia, to nonspecific headache, which may not be posterior and related or unrelated to other symptoms. In children with nonspecific headache, supportive evidence for diagnosis may be required before invasive procedures such as lumbar punctures are performed. Ultrasonography of the optic nerves (USON) is a noninvasive examination that can provide information about optic nerve changes, including those resulting from increased intracranial pressure. The applicability of USON in the diagnosis and follow-up of PTC was examined. STUDY DESIGN: Seventeen children were referred to our service because of a clinical suspicion of PTC, suggested by the presence of swollen optic nerve discs and/or headache, without clinical evidence of another cause. All had normal brain computed tomography and/or magnetic resonance imaging results before referral. Each child was examined with USON while in the supine position and with a 30-degree head tilt and underwent a lumbar puncture. USON was repeated on follow-up evaluation. RESULTS: The diagnosis of PTC was confirmed by lumbar puncture in 10 children and ruled out in 6 children. Overall, the USON results correlated with an increased opening pressure on lumbar puncture in 11 children. CONCLUSION: We noted an excellent correlation between the clinical results and the USON findings in PTC, and in many cases repeated lumbar punctures could be avoided. USON is more easily applied than a lumbar puncture, without the accompanying risks. It may be used as an indicator of increased intracranial pressure, as well as a follow-up tool. However, further studies are required before the accuracy of USON can be fully established.

Adolescent↗
PubMed1997

Visual pathway glioma: an erratic tumour with therapeutic dilemmas.

OBJECTIVE: Our experience in children with visual pathway glioma (VPG) was reviewed to delineate its clinical characteristics. DESIGN: The charts and imaging studies of 21 children with VPG who were followed up in our centre during the last 12 years were reviewed and summarised. RESULTS: VPG accounted for 13.1% of all brain tumours treated during this period. Sixty two per cent of the children with VPG had neurofibromatosis type 1 (NF-1). Among these, more than 60% were detected as part of routine work up. In some cases decreasing visual function preceded the appearance of the VPG on imaging studies. Tumour growth rate was markedly unpredictable. All treatment modalities employed led to tumour shrinkage and stabilisation for a variable period, but none was successful in totally eradicating the tumour. Complications were less severe after chemotherapy compared with radiotherapy. Three children died, none with NF-1, with a globular hypothalamic/chiasmatic tumour and accompanying electrolyte abnormalities. CONCLUSIONS: NF-1 is a favourable prognostic marker for VPG. Whenever possible a period of observation is necessary before treatment is initiated, during which time tumour size and visual function should be closely followed up; an untoward change in either of these is an indication for the start of treatment, preferably chemotherapy first. The combination of a globular hypothalamic/chiasmatic glioma and electrolyte abnormalities in a child without NF-1 are related to a poor prognosis.

Antibiotics, Antineoplastic↗
PubMed1996

Parental anxiety in febrile convulsions.

Febrile convulsions can cause extreme anxiety to parents. Factors associated with parental anxiety and its relief during and after admission of their child for simple febrile convulsions were studied by questionnaire. Of the 70 parents approached 46 responded (66%), all of whom reported feeling anxiety. Factors directly associated with parental anxiety were lack of knowledge regarding the management of seizures and the performance of a spinal tap or EEG. There was no correlation between a previous seizure in the same child or in a first-degree relative and a decrease in anxiety in the present seizure. Hospital admission of the child yielded no relief of anxiety in 27.3% and some relief in 34.1%. Staff activity was insufficient regarding parental instruction in handling the child in the event of a recurrence. About two-thirds of the parents wished to continue neurologic follow-up after discharge, and about one-half preferred that the child be treated with anticonvulsants. Our findings show that a very intensive effort is required to relieve parental anxiety after febrile convulsions. It is suggested that routine parental preventive education in this area be conducted in well-baby clinics.

Acute Disease↗
PubMed1995

Aniridia: recent achievements in paediatric practice.

Aniridia is a rare panocular disorder which primarily involves not only the iris, but also the retina, optic nerve, lens and cornea. Visual acuity deteriorates as a result of nystagmus, glaucoma, cataract, corneal opacities and retinal hypoplasia. Aniridia may appear as an isolated disorder, most often familial with autosomal dominance or sporadically in association with at least 12 syndromes. Both familial isolated and Wilms tumour, bilateral sporadic aniridia, genitourinary abnormalities and mental retardation syndrome-associated aniridia have been traced to a mutation of the PAX6 gene on band 11p13. Since genetic diagnosis of this disorder is already possible, counselling affected families should be preceded by karyotype studies and linkage analysis in familial cases of isolated aniridia. In sporadic cases of isolated aniridia or WAGR syndrome, we suggest that PAX6 mutation analysis be employed.

Aniridia↗
PubMed1995

Cerebral calcinosis and learning disabilities in hypoparathyroidism: a cause and effect or a coincidence?

A 12-year-old child is described in whom the presentation of learning difficulties and history of previous cataract removal led to a diagnosis of primary hypoparathyroidism. Cranial computed tomography revealed extensive calcinosis, previously described only in adults with long-standing hypoparathyroidism, and neuropsychologic evaluation revealed a marked symmetrical decrease in intellectual function. The possibility of the severe complication of brain calcinosis necessitates prompt diagnosis and treatment of hypoparathyroidism.

Brain Diseases↗
PubMed1995

Problems of differentiation between epilepsy and non-epileptic paroxysmal events in the first year of life.

Twenty two babies under 1 year old were referred for evaluation of suspected epileptic seizures. Nine were found to have epilepsy. In the other 13--all developing normally, aged up to 10 months--the spells were non-epileptic paroxysmal events (NEPE). They consisted of five patterns of movement: (1) eye blinking; (2) 'no' movements; (3) body posturing with head and arm jerks; (4) masturbation-like movements; and (5) myoclonic head flexion. The NEPE were present for a period of two weeks to seven months. Although some NEPE cannot be clinically differentiated from true epilepsy, in these infants at least four interictal EEGs were normal, the spells completely resolved after a relatively short period without antiepileptic treatment, and the infants continued to develop normally with no evidence of epilepsy during a follow up period of 28 to 38 months. This sample indicates that the frequency of NEPE in the first year of life may be high. Cautious clinical consideration, repeat EEGs and, when appropriate, a few weeks' observation are recommended. Awareness of these benign behavioural spells in this young age group is important, and parents can be reassured. Nevertheless, the spells may illustrate a 'foggy frontier' between NEPE and epilepsy. The lack of evidence for any other disease process in affected infants, as well as the disappearance of the NEPE without any intervention, indicates that a maturational process may be involved.

Diagnosis, Differential↗
PubMed1995

'Complicated migraine-like episodes' in children following cranial irradiation and chemotherapy.

Neurologic sequelae may occur months to years after cranial irradiation. The site of primary damage is probably the vascular endothelium. Over a 2.8-year period, four children with brain tumors, a mean of 11 years of age at diagnosis (range, 6.5 to 15.5 years), had new onset of severe intermittent unilateral headaches associated with nausea, episodic visual loss, hemiparesis, aphasia, or hemisensory loss. The headaches lasted 2 to 24 hours. All patients had previously received whole-brain (2,400 to 3,600 cGy) and additional local boost (1,800 to 3,100 cGy) cranial irradiation, as well as cisplatin-, lomustine-, and vincristine-containing chemotherapy regimens. Symptoms began 1.2 to 2.8 years after the diagnosis, when all had stable disease and were off treatment. MRI studies were unchanged, and CSF cytology, EEGs, echocardiograms, and magnetic resonance angiograms were normal in all. Cerebral angiograms, performed in three children, were normal but led to severe headaches and neurologic deficits (hemiparesis in one and visual loss in two) that resolved after 24 to 48 hours. Response to antimigraine and antiplatelet medications was variable. We conclude that (1) "complicated migraine-like episodes" may occur in children after cranial irradiation and chemotherapy as a sequela of therapy; (2) these headaches may not be the harbinger of impending strokes, severe intracranial vasculitis, or tumor recurrence; and (3) while cerebral angiography may be useful in differential diagnosis, it may cause transient worsening of symptoms.

Adolescent↗
PubMed1992

Confusion as the presenting manifestation of vertebral osteomyelitis: a case report.

A 44-year-old patient presented with increasing confusion. He was first diagnosed as having intermittent pressure hydrocephalus but a further evaluation showed CSF pleocytosis and hypoglycorrhachia. Five weeks later, his physical examination was unrevealing. Nuclear imaging techniques were conflicting, with negative gallium- and indium-labelled white blood cells scans but a Tc scan pointing towards a vertebral infection. A well-demarcated lesion in the T9 vertebral body, demonstrated by CT scan, confirmed the diagnosis of vertebral osteomyelitis. Although we were unable to recover the causative organism, antibiotic treatment for presumed staphylococcal osteomyelitis resulted in full recovery. This case indicates that vertebral osteomyelitis may cause significant meningeal inflammation even in the absence of epidural or subdural abscess. We recommend that in patients with meningitis without a clear etiology vertebral osteomyelitis should be considered and pursued with CT scannings of the vertebrae, a procedure that can yield positive findings even when other scanning modalities are negative.

Adult↗
PubMed1991

Early invasive CNS aspergillosis. An easily missed diagnosis.

A 46-year-old woman with non-Hodgkin's lymphoma presented with a new onset of seizures. A cranial CT was interpreted as normal. Eight months later, she presented with changed mental status and leg weakness, and a repeat CT scan showed multiple ring-enhancing lesions close to the left frontal sinus, with mass effect. A review of the previous CT scan showed a very small area of sinusitis as well as a small ring-enhancing lesion contiguous to it. A short course of intravenous steroids markedly relieved her symptoms, and a stereotactic biopsy confirmed Aspergillus fumigatus to be the cause of the infection. She was successfully treated with Amphotericin B. Central nervous system (CNS) aspergillosis is a potentially fatal disease. The therapeutic success in this case was related to a high index of suspicion at her second presentation. As the early signs of infection might be subtle and easily missed, patients at high risk for opportunistic infection should be studied carefully when new onset of CNS symptoms occur. Early initiation of therapy should improve the prognosis in such cases.

Aspergillosis↗
PubMed1991

Jitteriness beyond the neonatal period: a benign pattern of movement in infancy.

Jitteriness is a frequent, well-recognized phenomenon in neonates. Its occurrence in early infancy, beyond the neonatal period, is much less documented. Thirty-eight full-term infants who were jittery after 6 weeks of age were followed until the jitteriness disappeared and then reevaluated at the age of 3 years. The jittery movements had already been observed during the neonatal period in 16 babies but not before 6 weeks of age in 22. In 34 infants (89%), 1- and 5-minute Apgar scores were 9 or 10. At the initial examination, a mildly increased muscle tone was found in 12 infants, and hyperactive deep-tendon reflexes were found in 19. The jitteriness resolved at a mean age of 7.2 +/- 3.4 months. At 3 years, 92% of the infants had a normal neurodevelopmental examination, while in the rest only minor, transient disturbances were found. This study indicates that jitteriness as a sole presentation in infants beyond the neonatal period is a benign phenomenon, associated with an excellent prognosis. Its etiology is unknown but seems most likely to be related to a maturational process within the central nervous system.

Apgar Score↗
PubMed1990

New findings in a patient with Dubowitz syndrome: velopharyngeal insufficiency and hypoparathyroidism.

We report on a boy with Dubowitz syndrome and hypoparathyroidism from which he recovered, only to redevelop it at 6 years. He also had a submucous cleft palate and cineradiographic studies showed velopharyngeal insufficiency. Although a submucous cleft palate is a well-known manifestation of Dubowitz syndrome, velopharyngeal insufficiency has not been previously described.

Abnormalities, Multiple↗
PubMed1990

Central nervous system vasculitis after chickenpox--cause or coincidence?

A 7.5 year old boy, known to have a seizure disorder, presented with an infarct in the left middle cerebral artery territory, 10 weeks after severe chickenpox. Immunofluorescent antibody titre to the varicella zoster virus in the cerebrospinal fluid was 1:32. Cerebral angiography showed evidence of focal vasculitis. He presented again seven months later with an acute exacerbation of seizures. Magnetic resonance imaging of the brain showed an old posterior extension of the infarct, but a repeated angiography demonstrated an improvement in the vasculitic process. Cerebrospinal fluid antibody titre was again 1:32. Although this may have been an unfortunate coincidence, a possible association between chickenpox and vasculitis, similar to that reported with herpes zoster, and with potentially significant clinical implications, should be considered. As a definite proof can be obtained only by a brain biopsy, however, which is generally not indicated in such cases, only additional clinical reports can lead to delineation of this association as a definite entity.

Brain Diseases↗