William E. Bell

Idiopathic Intracranial Hypertension in Children: The Iowa Experience

Idiopathic intracranial hypertension is an uncommon condition in children and is seen only about once a year in a large referral hospital. Sex incidence is equal, and obesity is a feature in about one third of patients. This article reviews the 30-year experience in an institution where idiopathic intracranial hypertension in adults has been extensively studied. Symptomatic causes of intracranial hypertension and both medical and surgical therapeutic options are reviewed. (J Child Neurol 1994;9:144-149).

Neuroradiographic abnormalities in congenital cytomegalovirus infection.

To determine the spectrum of neuroradiographic abnormalities associated with congenital cytomegalovirus (CMV) infection, CT brain scans of 15 infants with symptomatic infection were reviewed. The initial CT scans were abnormal in 13 patients. Abnormalities included intracranial calcifications, cortical atrophy, ventricular enlargement, subdural effusions, porencephaly and polycystic encephalomalacia. Intracranial calcifications were present in 33% of the infants. In addition, three of the 15 infants developed progressive hydrocephalus which required ventriculoperitoneal shunt placement. These cases illustrate that congenital CMV infection causes a variety of structural CNS lesions and suggest that progressive hydrocephalus may be a relatively common consequence of symptomatic congenital CMV infection.

Brain tumors occurring before 1 year of age: a retrospective reviews of 22 cases in an 11-year period (1977-1987).

Congenital brain tumors have been reported infrequently and their management remains ill defined. An 11-year review (1977-1987) of all children with brain tumors with the onset of symptoms before 1 year of age was completed. Twenty-two children with the following histological diagnoses were treated: astrocytoma (7 patients), primitive neuroectodermal tumor (6 patients), papilloma or carcinoma of the choroid plexus (3 patients), malignant teratoma (2 patients), dermoid tumor (2 patients), embryonal rhabdomyosarcoma (1 patient), and chloroma (1 patient). Fifteen tumors were supratentorial in location, and 7 were infratentorial. Initial symptoms were hydrocephalus (32%), focal neurological deficit (23%), asymptomatic increase in head circumference (18%), failure to thrive (14%), and seizures (4.5%). The goal of treatment was a radical excision when possible, with primary chemotherapy in the last 6 years of the review period. Radiation therapy was the adjunct to surgery in the initial 5-year period. All patients with papillomas of the choroid plexus and dermoid lesions underwent a total resection with no recurrence. All 7 astrocytomas were supratentorial, with 6 occurring in the diencephalon. Five of the seven patients with astrocytomas survived more than 5 years. The 6 primitive neuroectodermal tumors were located equally between the supra- and infratentorial spaces. Four of the 6 infants with these tumors received chemotherapy (2 received chemotherapy alone; 2 received chemotherapy and radiation therapy) and are tumor free 2 to 9 years later. A fifth child received radiation therapy alone early in the series and survived only 4 months. The family of the other child refused adjunctive treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

MRI in children with postinfectious disseminated encephalomyelitis.

Acute disseminated encephalomyelitis, an inflammatory and demyelinating disorder of central nervous system white matter, typically occurs following childhood viral infections. Although CT may demonstrate abnormalities, many children have normal CT studies in spite of widespread neurologic abnormalities. We report a series of five patients with the typical clinical presentation of disseminated encephalomyelitis who were studied using magnetic resonance imaging (MRI). In each case the children presented with progressive subacute neurologic abnormalities including headache, diplopia, ataxia, hemiparesis, seizures, dysarthria, and/or coma. CT was nondiagnostic. MRI clearly demonstrated multifocal white matter lesions of the cerebrum, brainstem, and cerebellum which corresponded to clinical signs. The patients improved dramatically with corticosteroid therapy. MRI showed progressive resolution of multifocal lesions in conjunction with clinical improvement.

ETIOLOGY OF LATERAL RECTUS PALSY IN INFANCY AND CHILDHOOD

The etiologies of lateral rectus palsy in 132 infants and children seen over a period of 22 years in a university medical center were retrospectively reviewed and compared with similar reports in the literature. Unlike most reports, which lump children with adults, this study focuses on childhood etiologies of lateral rectus palsy. In contrast to the only two studies in the literature on childhood lateral rectus palsy, which reflect a purely ophthalmologic perspective, this study reflects experiences of pediatric neurology and pediatric neurosurgery, as well as ophthalmology. Most lateral rectus palsies were unilateral, almost equally distributed between right and left sides. Trauma, tumor, and congenital etiologies were the most prevalent. The relative frequency of each of these etiologies varies by service (neurology, neurosurgery, ophthalmology). In 10.6% of patients, etiology could not be determined. The majority of patients had an isolated lateral rectus palsy. Association of lateral rectus palsy with other cranial nerve palsies and/or long tract signs characterized trauma and tumor. The majority of tumors were primary and infratentorial. A small number of patients had benign recurrent lateral rectus palsy. (J Child Neurol 1992;7:295-299).

Accelerated myelination in early Sturge-Weber syndrome demonstrated by MR imaging.

Magnetic resonance imaging of the brain in two infants with Sturge-Weber syndrome has demonstrated a pattern of accelerated myelination in the abnormal cerebral hemisphere. The extent of myelination was most apparent on the T1-weighted inversion recovery sequence while the T2-weighted images demonstrated concomitant changes in hydration of the brain. We propose an explanation for this finding based on cerebral ischemia underlying the leptomeningeal angioma.

Posterior Fossa Hemorrhage in the Term Neonate

Posterior fossa hemorrhage (PFH) in the newborn term infant has been infrequently reported, and before computed tomographic (CT) scanning most were identified at postmortem examination. The origin and causes of PFH in the term neonate differ from those in adults. The main possible causes are: (a) tentorial and falx disruption with avulsion of bridging veins due to mechanical compression and distortion of the head during the birth process, resulting in posterior fossa subdural collections and vermis hematoma, and (b) occipital osteodiastasis associated with cerebellar hemisphere laceration, with intracerebellar hematoma and subdural collections. Three full-term neonates with PFH were operated on with good outcome. Both of the possible causes contributed to the hemorrhage. Review of the literature to date has led to the following conclusions: (a) PFH in the term infant is frequently associated with breech delivery, forceps instrumentation, and prolonged labor with cranial molding. (b) Massive PFH is associated with dural sinus laceration, rupture of the vein of Galen, or rupture of hematoma into the subdural space. Symptoms may be delayed up to 96 hours in cases where there is a small continuing hemorrhage. (c) CT scanning with reconstructions is essential to provide precise localization of the hemorrhage and thus avoid errors in interpretation. (d) Prompt removal of PFH improves survival and decreases sequelae. (e) Shunting procedures are necessary in 36% of the cases.

Chloral Hydrate Sedation As a Substitute for Examination Under Anesthesia in Pediatric Ophthalmology

We examined more than 300 children as outpatients with orally administered chloral hydrate sedation during the past three years; 175 of them have been examined in the last year. Without chloral hydrate sedation, examination under anesthesia would have been necessary or the results would have been less than satisfactory. Orally administered chloral hydrate has a wide margin of safety and is most appropriate for office or clinic use. It may also allow accurate measurement of the intraocular pressure in infantile glaucoma.

Epidermal Nevus Syndrome: Subgroup With Neuronal Migration Defects:

Epidermal nevus syndrome is one of the sporadic congenital hamartoses in which neurologic abnormalities have been frequently reported. We report two cases with severe primary brain involvement, seizures, mental retardation, and facial hemihypertrophy. We emphasize the superiority of magnetic resonance imaging over other radiographic studies in outlining the primary central nervous system anomalies associated with this syndrome. Although attempts were made to distinguish between several variants of epidermal nevus syndrome, it is clear that these are one entity. Proteus syndrome, encephalocraniocutaneous lipomatosis, and epidermal nevus syndrome have several overlapping phenotypic features. We suggest that they represent a phenotypic continuum, which in turn suggests a common pathogenetic process. While the cause of these syndromes is unknown, observations point to a somatic mutation leading to variable patterns of mosaicism. (J Child Neurol 1992;7:29-34).

Myelinoclastic Diffuse Sclerosis (Schilder's Disease): Report of a Case and Review of the Literature:

The clinical, neuroimaging, and neuropathologic features of Schilders disease in a 17-year-old girl are presented and compared to 11 well-documented cases reported since 1912. The evolution of knowledge about Schilders disease and the confusion in nomenclature are reviewed. Signs and symptoms in this case and others reported in the literature are nonspecific and may mimic mass lesions. Neuroimaging studies also may mimic brain tumor or abscess; however, the absence of significant edema, the irregular and incomplete ring enhancement, the discrepancy between size of the lesions and the associated mass effect, and the absence of other lesions elsewhere in the brain may help differentiate Schilders disease from neoplasm, infection, and other demyelinating lesions. Although frozen sections of these lesions are often interpreted as astrocytoma, the inflammatory, primarily histiocytic, nature of Schilders disease is more easily recognized in paraffin-embedded material. Unique features of this case include multiple unilateral lesions and the cyst-like degeneration present in both lesions. Multiple lesions in Schilders disease are characteristically bilateral. The examination of aspirated fluid is the first such report in Schilders disease. The limitation of multiple lesions in our case to one hemisphere calls for reexamination of the restrictive 1985 criteria of Poser for the diagnosis of Schilders disease. (J Child Neurol 1994;9:398-403).