Hypomelanosis of Ito. Wood's light and magnetic resonance imaging as diagnostic measures.

Hypomelanosis of Ito is an uncommonly reported neurocutaneous syndrome with a peculiar pattern of swirling hypopigmentation. The skin markings in the lightly pigmented individual are not easily visualized in regular light but can be readily seen with the use of Wood's light. We describe a 7-month-old female infant with this disorder who presented with intractable convulsions. Computed tomography showed enlargement of the left cerebral hemisphere but was otherwise not definitive. Abnormalities of neuronal migration were demonstrated with magnetic resonance imaging that could not be shown by computed tomography.

Allescheria (Petriellidium) boydii brain abscess in a child with leukemia.

A 3-year-old boy with acute lymphoblastic leukemia developed a right frontal lobe abscess from which Allescheria (Petriellidium) boydii was cultured. The mycotic infection complicating his underlying disease appeared to be confined to the brain. Surgical drainage and therapy with amphotericin B were followed by eventual recovery with no neurologic deficits.

Arachnoid cysts in children.

Twenty-seven cases of intracranial arachnoid cysts in children aged 1 to 16 years have been studied with attention to diagnosis, pathologic findings, and surgical treatment. In nine patients, the cyst was located in the posterior fossa. In eight patients, there were other CNS or other organ system abnormalities. No specific symptom complex can be ascribed to arachnoid cysts in that the clinical manifestations are the result of their size and location, secondary complications, and remote effects. This is illustrated in the ten cases reported.

Magnetic resonance imaging of the spine in children.

We reviewed the magnetic resonance imaging (MRI) scans of the spine of 42 children who had neurologic signs compatible with lesions of the spinal cord. Twenty-three of the children had abnormalities identified by MRI. The spectrum of abnormalities included posttraumatic lesions, tumor, tethered cord, and syringohydromyelia. Tethered cord with or without lipoma and syringohydromyelia were the most common findings, affecting six and four children, respectively. Thirteen children studied by MRI also underwent conventional metrizamide myelography and/or computed tomography. In 12 cases, the findings of MRI were comparable to those of myelography and computed tomography. These observations indicate that MRI effectively detects lesions of the spinal cord in children. Because MRI can be performed on an outpatient basis and avoids the risks of metrizamide myelography, we conclude that MRI should be considered to be the preferred screening technique for children with suspected spinal cord disorders.

Cerebral hemiatrophy, hypoplasia of internal carotid artery, and intracranial aneurysm. A rare association occurring in an infant.

A 4-month-old child with a very rare association of primary (congenital) left cerebral hypoplasia, hypoplasia of the left internal carotid artery, and giant anterior communicating artery aneurysm was seen. The aneurysm was detected and treated before rupture. We briefly reviewed the literature on agenesis (hypoplasia) of the internal carotid artery, intracranial aneurysms in childhood, and primary cerebral hypoplasia. It is proposed that cerebral arteriography, cranial computed tomography, or magnetic resonance imaging be performed in all children with primary cerebral hemihypoplasia to establish an etiologic diagnosis and, more importantly, to detect and treat possible associated intracranial aneurysms before they become symptomatic.

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.

Evaluation of bromfenac and ibuprofen for pain after orthopedic surgery.

STUDY OBJECTIVES: To determine the relative analgesic potency and adverse effect liability of bromfenac 25, 50, and 100 mg, and ibuprofen 200 and 400 mg in the treatment of postoperative pain after orthopedic surgery. DESIGN: Randomized, double-blind, single-dose, parallel-group relative potency assay with evaluations at 30 minutes and then at hourly intervals for up to 6 hours. SETTING: Two wards of the orthopedic surgery department at the Centralsjukhuset (Central Hospital) in Karlstad, Sweden. PATIENTS: Two hundred inpatients with steady, moderate or severe pain within 72 hours after orthopedic surgery. INTERVENTIONS: Patients received a single oral dose of bromfenac 25, 50, or 100 mg, or ibuprofen 200 or 400 mg, when they experienced steady, moderate or severe pain that, in their opinion, required an analgesic. Using a self-rating record, subjects rated their pain and its relief for 6 hours after medicating. MEASUREMENTS AND MAIN RESULTS: The study was a valid relative potency assay with estimates of bromfenac's potency relative to ibuprofen ranging from 10.9 (nurse's global evaluation) to 16.7 (sum of hourly analog pain intensity difference scores). That is, 11-16 times the dose of ibuprofen must be administered to equal the analgesic effect of bromfenac. Patients who had eaten breakfast or lunch within 60 minutes before or 30 minutes after receiving the study medication ("fed" patients) had lower efficacy scores than those who had not ingested food within these time constraints before or after receiving the study medication ("fasted" patients). Furthermore, patients who had eaten before receiving the study medication had significantly lower efficacy scores than those who had eaten after receiving the study medication. CONCLUSIONS: The relative potency of the analgesic effect of bromfenac to ibuprofen is 11-16.7 in patients with pain after orthopedic surgery. Fed patients may have lower analgesic efficacy than fasted patients. Adverse effects for both bromfenac and ibuprofen were transient and consistent with the pharmacologic profiles of the drugs.

Evaluation of two opioid-acetaminophen combinations and placebo in postoperative oral surgery pain.

STUDY OBJECTIVE: To determine the relative analgesic potency and adverse effect liability of hydrocodone bitartrate 7.5 mg with acetaminophen 500 mg, codeine phosphate 30 mg with acetaminophen 300 mg, and placebo in the treatment of pain following oral surgery. DESIGN: Randomized, double-blind, single-dose, placebo-controlled, parallel-group study with self-ratings at 30 minutes and then at hourly intervals from hour 1 to hour 6. SETTING: Private, oral surgery practice sites. PATIENTS: Three hundred twenty-four outpatients with moderate or severe pain after the surgical removal of impacted third molars were selected. One was lost to follow-up and 32 did not need an analgesic; 232 patients had valid efficacy data. INTERVENTIONS: Patients were treated with a single oral dose of hydrocodone bitartrate 7.5 mg with acetaminophen 500 mg, codeine phosphate 30 mg with acetaminophen 300 mg, or placebo when they experienced steady, moderate or severe pain that, in their opinion, required an analgesic. Using a self-rating record, subjects rated their pain and its relief for 6 hours after medicating; estimates of peak and total analgesia were derived from these subjective reports. MEASUREMENTS AND MAIN RESULTS: This study was a valid analgesic assay. Both active treatments were significantly superior to placebo for all measures of analgesic efficacy. The hydrocodone-acetaminophen combination was significantly superior to the codeine-acetaminophen combination for total pain relief and the number of evaluations with 50% relief. Both active treatments manifested an analgesic effect within 30 minutes; the effect persisted for 5 hours for the codeine combination and 6 hours for the hydrocodone combination. Adverse effects were transient, consistent with the pharmacologic profiles of opioids, and none required treatment. CONCLUSIONS: A slight advantage in analgesic efficacy was demonstrated in this single-dose study for the hydrocodone-acetaminophen combination. Repeat-dose studies, however, should be conducted to determine the clinical significance of the difference in analgesic effect of these opioid combinations.

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.

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)

Detection of heterotopic gray matter in children by magnetic resonance imaging.

Heterotopic gray matter results from abnormal brain development and is a recognized focus of seizures. It may be associated with mental retardation and/or severe malformations of the brain. Three patients with heterotopia of gray matter were identified by magnetic resonance imaging (MRI). CT failed to detect the heterotopic gray matter in each case. One child was referred for removal of a neoplasm based on CT studies until MRI demonstrated the developmental nature of his condition. One infant had severely dysplastic left cerebral hemisphere associated with heterotopic gray matter and the syndrome of Hypomelanosis of Ito. All three children suffered from seizures and/or mental retardation. MRI provided important information in the management of each case and appears to be the imaging method of choice in evaluating children with seizures or retardation for heterotopic gray matter in the brain.

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.

Tests for juvenile myasthenia gravis: comparative diagnostic yield and prediction of outcome.

The clinical picture, family history, laboratory data, treatment modalities, and outcome in 27 juvenile myasthenics seen over a 25-year period are presented. Onset was after 10 years of age in the majority of patients. Half presented with ocular signs, the other half with generalized-onset myasthenia. Half of those with ocular-onset myasthenia progressed to generalized myasthenia. The female-to-male ratio was 3.5:1. Myasthenia gravis was reported in the mother of one patient. Ptosis was the most common presenting sign. It was unilateral at onset in 33% of patients and remained unilateral in 11%. Pharmacologic tests (neostigmine methylsulfate and edrophonium chloride) were positive in 92% of patients. Serology was positive in 63%, whereas repetitive nerve stimulation was positive in 33% when distal nerves were stimulated and in 66% when proximal and distal nerves were stimulated. Seropositivity tended to increase with generalization of the myasthenic process. No statistically significant difference in seropositivity was noted between males and females. Anti-striated muscle antibodies were detected in two patients, neither of whom had thymoma. The yield of repetitive stimulation increased with generalization of the myasthenic process and when proximal nerves were stimulated. No statistically significant difference was noted in the decremental response between seropositive and seronegative patients. The majority of ocular myasthenics were treated with pyridostigmine bromide monotherapy. With generalization of the myasthenic process, other modes of medical and surgical therapies were needed. All patients given corticosteroids ultimately underwent thymectomy. The mean age at presentation and the duration of symptoms at presentation were longer in thymectomized patients than in those without thymectomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Computed tomography and magnetic resonance imaging of the brain in Hurler's disease.

Computed tomographic (CT) and magnetic resonance imaging (MRI) scans of the brain in five patients with Hurler's disease are described and compared to the few available reports in the literature. Computed tomographic scans revealed low attenuation areas in the centrum semiovale and peritrigonal white matter. Ventriculomegaly was not a prominent feature in our patients, compared to those previously reported. In two patients, CT were normal. The most prominent magnetic resonance imaging abnormalities were the presence of radially oriented cystic areas in the centrum semiovale, peritrigonal white matter, corpus callosum, and pericallosal region. Magnetic resonance imaging abnormalities were present in all patients, even when CT scans were normal. Abnormalities on CT and MRI scans tended to be more prevalent in the posterior regions. Magnetic resonance imaging proved to be a more reliable imaging method in Hurler's disease. T1-weighted images delineated the cystic areas more clearly, whereas T2-weighted images were more sensitive in detecting small white-matter abnormalities. Magnetic resonance imaging abnormalities correlated well with known neuropathologic alteration in this disease. It is suggested that the cystic areas seen on MRI correspond to perivascular lacunae seen in histopathologic material.

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.

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.

Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature.

The clinical, neuroimaging, and neuropathologic features of Schilder's disease in a 17-year-old girl are presented and compared to 11 well-documented cases reported since 1912. The evolution of knowledge about Schilder's 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 Schilder's 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 Schilder's 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 Schilder's disease are characteristically bilateral. The examination of aspirated fluid is the first such report in Schilder's 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 Schilder's disease.

Aqueductal stenosis and neurofibromatosis: a rare association.

Three cases with rare association of neurofibromatosis, hydrocephalus, and aqueductal stenosis seen at the University of Iowa during the past 20 years are presented. The literature on nontumoral hydrocephalus and aqueductal stenosis in neurofibromatosis is reviewed. Possible explanations for the association of aqueductal stenosis and neurofibromatosis are discussed. It is concluded that aqueductal stenosis, though not common, should nevertheless be considered in the differential diagnosis of hydrocephalus in neurofibromatosis.

Acquired immobility of the right arm.

The occurrence of an asymmetrical motor dysfunction is an event that frequently results in referral to the pediatric neurologist. The differential diagnosis is not extensive but is somewhat larger than superficial evaluation might indicate. This 4-year-old boy developed intermittent painful motor dysfunction of the right arm over a 1-year period of time resulting from an unusual cause of unilateral motor dysfunction.

Headaches of recent onset and long-term learning disability.

In the evaluation of headaches, history is everything! Usually. However, sometimes the headaches are associated with unrelated conditions simply because of their frequency. The evaluation of a child with a learning disability is also a common exercise and the trick, with both conditions, is to recognize the occasional patient who warrants further evaluation.