Diffuse intrinsic brainstem disease with neurologic deterioration: not what it seemed.
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Adolescent↗
Biomedical subjects
A Shuper
Publications and source records attributed to A Shuper.
Delayed post-traumatic visual loss: a clinical dilemma.
A 16-year-old female presented with unilateral blindness in her right eye 2 months after blunt head trauma. Optic nerve edema was demonstrated by funduscopic examination, ultrasonography, and magnetic resonance imaging and failed to respond to medical treatment. Delayed post-traumatic blindness may be a severe complication of head trauma. The late appearance leads to delayed diagnosis and resulting unresponsiveness to treatment. Patients who experience head trauma that could involve the optic nerves should undergo ultrasonography of the optic nerves. An abnormal finding should be followed by an intensive evaluation to determine possible damage.
Adolescent↗
Transient opercular syndrome: a manifestation of uncontrolled epileptic activity.
Newly appearing oral motor apraxia should alert physicians to the possibility of an organic lesion in the operculum, the perisylvanian cortex. A 6 1/2-year-old boy who is being followed for complex partial seizures with secondary generalization failed to respond to carbamazepine and then to the addition of sodium valproate. The further addition of lamotrigine stopped the seizures but the patient presented with a new onset of opercular syndrome, manifested by severe oral motor apraxia with difficulties in chewing and swallowing and speech. MRI study of the brain revealed no abnormalities. The opercular syndrome resolved completely when the lamotrigine was replaced with phenobarbital, and did not recur during the follow-up period of 2 years. The EEG, however, continued to be abnormal. We conclude that opercular syndrome may be a manifestation of abnormal localized electrical activity at the operculum, even in the absence of an organic lesion on imaging studies, and it may be a marker for an epilepsy which is not easily controlled.
Anticonvulsants↗
Visual pathway tumors and hydrocephalus.
The authors evaluated the impact of hydrocephalus on the clinical picture of children with visual pathway tumor (VPT) with or without neurofibromatosis (NF). Charts of children with VPT treated in the authors' center since 1985 were retrospectively reviewed, and those with hydrocephalus were selected and summarized. Thirty-five children with VPT were found, of whom 20 had NF. Hydrocephalus was found in 4 children with NF (20%) and in 5 without NF (33.3%). In 6 of the children, ventricular dilatation with signs of acute increased intracranial pressure already existed at the time of diagnosis and the hydrocephalus was shunted at this time. In the other 3 children, all with NF, the hydrocephalus resulted from slowly developing aqueductal stenosis, leading in 2 to severe visual acuity deterioration. The results suggest that in children with VPT and NF, hydrocephalus, and especially hydrocephalus resulting from aqueductal stenosis, is more frequent than in the general population of NF patients, and less frequent than in VPT patients without NF. The possibility of the indolent development of hydrocephalus should be borne in mind while following children with NF. The optic nerve, when already involved with a glioma, is more vulnerable to increased pressure. Thus, in children with VPT and NF, any ventricular dilatation should lead to a consideration of early shunting.
Child↗
The paediatrician and the rabbi.
OBJECTIVES: During recent decades, rabbis in Israel have been playing an increasing role in the consultation of patients or their families on medical issues. The study was performed to determine the attitude of physicians to rabbinical consultation by parents of sick children for purposes of basic medical decision making. DESIGN AND SETTING: A questionnaire was prepared which contained questions regarding physicians' reactions to specific medical situations as well as their demographic data. The study participants included all the available physicians who were employed in the study period at one tertiary medical centre in Israel, which is not associated with any religious organisation. The questionnaire was presented personally to all of the physicians who were available for the study. RESULTS: Between 63% and 77% of the respondents were accepting of rabbinical consultation in regard to medical decisions. Nevertheless, in cases of divergence from accepted medical practice and in emergencies, almost all stated they would take measures to resist the rabbi's advice. This attitude did not correlate with the physician's age, religious status or experience in medicine. CONCLUSIONS: Israeli physicians respect rabbis' suggestions in the area of medical decision making, though they would not let a rabbi's advice interfere with their decisions if they believed the rabbi's opinion went against medical need. In order to prevent an untoward effect of the rabbinical involvement in medicine, rules should be set to establish norms for rabbi-physician collaboration.
Attitude of Health Personnel↗
Subacute central nervous system degeneration in a child: an unusual manifestation of ifosfamide intoxication.
A 5-year-old child with desmoplastic small round-cell tumor was treated with a protocol of very-high-dose, short-term chemotherapy, containing HD-CAV (cyclophosphamide, doxorubicin, vincristine, and mesna), ifosfamide, and etoposide. Two days after the initiation of ifosfamide, he exhibited new-onset lethal encephalopathy manifested by subacutely progressive cerebellar and then temporal and frontocortical degeneration leading to a vegetative state and eventually to death. A full work-up, including brain biopsy, was negative, excluding infections and metabolic or vascular causes. Ifosfamide is known to be capable of causing acute encephalopathy that can be severe but is generally reversible. This child showed a very atypical progressive, lethal course of ifosfamide toxicity. The possibility of this complication should be considered when high-dose ifosfamide treatment is planned for children.
Antineoplastic Combined Chemotherapy Protocols↗
Methotrexate treatment protocols and the central nervous system: significant cure with significant neurotoxicity.
Methotrexate can influence the central nervous system through several metabolic toxic pathways. These effects can be categorized as immediate, acute to subacute, or chronic neurologic syndromes. The acute to subacute syndrome occurs frequently in acute lymphoblastic leukemia treatment protocols, generally manifesting with focal neurologic signs and changes seen on magnetic resonance imaging and single photon emission computed tomography. While in some patients the neurotoxicity is transient and benign and allows for continuation of chemotherapy, in others it can be quite severe and debilitating, leading to permanent neurologic deficits. The need to modify the treatment protocols when neurotoxicity appears is not fully established. It is also unknown whether the use of sufficient amounts of leucovorin can overcome the toxic effects of the drug.
Adolescent↗
Secondary enuresis: post-traumatic stress disorder in children after car accidents.
BACKGROUND: In our experience, secondary enuresis nocturna is a common complaint among children after a motor vehicle accident. However, as these children are often brought for examination as part of an insurance compensation claim, this complaint is not always reliable. OBJECTIVE: To describe a series of children in whom secondary enuresis occurred after a motor vehicle accident. METHODS AND RESULTS: Five children were brought to our clinic for evaluation of secondary nocturnal enuresis. Review of past history revealed a car accident preceding the onset of the enuresis. All but one had additional behavioral symptoms typical of post-traumatic stress disorder. Four children had evidence of head trauma, and one had psychological but no physical trauma. CONCLUSIONS: Nocturnal enuresis can occur after a motor vehicle accident due either to purely psychological trauma or organic head trauma. While nocturnal enuresis is generally attributed to organic causes, psychological mechanisms also play a significant role.
Accidents, Traffic↗
Periventricular brain heterotopias in a child with adrenocortical insufficiency, achalasia, alacrima, and neurologic abnormalities (Allgrove syndrome).
We describe a previously unreported finding of periventricular heterotopias in a brain magnetic resonance imaging (MRI) study, in a girl with adrenocortical insufficiency, alacrima, achalasia, and neurologic deterioration (Allgrove syndrome). This finding could indicate that the underlying mechanism in this syndrome can be traced to the first half of fetal life and also might cause abnormal neuronal migration. This disorder recently has been linked to chromosome 12q13. There could be several explanations for the clinical heterogeneity in this syndrome: a contiguous gene syndrome involving multiple genes, including one whose deletion causes heterotopias, or a deficiency of a gene for a neurotrophic factor active during pre- and postnatal life and responsible for both migration and survival of neurons could be the cause. The identification of the responsible gene(s) will lead to further understanding of this multisystem disorder.
Adrenal Insufficiency↗
[Future treatment modalities in Duchenne muscular dystrophy].
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Animals↗
Diffuse brain stem gliomas. Are we improving outcome?
We reviewed our experience with diffuse brain stem glioma (dBSG) to evaluate whether any improvement of outcome had occurred in our patients over the years. Of the 24 children referred to our department with suspected dBSG from 1981 to 1997, 5 had a different final diagnosis based on the clinical course. Mean survival in the remainder was 16+/-9.8 months from diagnosis. Survival increased with a longer interval from onset of symptoms to diagnosis (12.9+/-9.0 months with an interval of 1-4 weeks; 19.50+/-10.8 months with a longer interval). Visual symptoms at presentation were associated with a poorer prognosis. Survival was better in the 3- to 5-year age group (at diagnosis). Overall, a trend toward a slight improvement in survival was seen over the years, which we presumptively attribute to the introduction of intensive chemotherapy for these patients. We suggest that chemotherapy may be important in the management of dBSG until a better modality is found.
Adolescent↗
Familial hemophagocytic lymphohistiocytosis: improved neurodevelopmental outcome after bone marrow transplantation.
A child with familial hemophagocytic lymphohistiocytosis (HLH) underwent allogeneic bone marrow transplantation (BMT) at age 5 months. At that time he showed delayed psychomotor development, and computed tomography revealed diffuse calcification. After BMT, a gradual neurodevelopmental normalization was observed. The potential ability of BMT to reverse neurodevelopmental deterioration in HLH should be considered.
Bone Marrow Transplantation↗
Myopathic changes as a paraneoplastic sign in childhood acute lymphoblastic leukemia.
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Child, Preschool↗
Metastatic brain involvement in Ewing family of tumors in children.
OBJECTIVE: To evaluate the incidence and clinical characteristics of CNS involvement in Ewing family of tumors (EF) in children. METHODS: Chart reviews of children with EF treated in our center from 1972 to 1997. Clinical and imaging data regarding possible CNS involvement were collected. RESULTS: During this 25-year period, 80 children with EF were treated. Intracranial involvement was found in eight (10%) children: the brain was involved in seven children (8.8%) and a retro-orbital metastasis without parenchymal brain involvement was noted in one child. Metastases were localized intrahemispherically, or in the cerebellum or the basal ganglia. Intracranial spread was hematogenous in five children and by contiguous spread from the skull in three children. Intracranial involvement was diagnosed 1.3 to 11 years from initial presentation. Seizures and hemiparesis were the main neurologic complications. CONCLUSIONS: The rate of parenchymal brain involvement in our patients with EF was 8.8%. Spread was mainly hematogenous. Substantial morbidity was associated with CNS disease, which appeared in most patients late in the course of disease.
Adolescent↗
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↗
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↗
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↗