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Biomedical subjects

J Nadell

Publications and source records attributed to J Nadell.

17 recordsLinked to original sources

Intrathecal baclofen for management of spastic cerebral palsy: multicenter trial.

Intrathecal baclofen infusion has demonstrated effectiveness in decreasing spasticity of spinal origin. Oral antispasticity medication is minimally effective or not well tolerated in cerebral palsy. This study assessed the effectiveness of intrathecal baclofen in reducing spasticity in cerebral palsy. Candidates were screened by randomized, double-blind, intrathecal injections of baclofen and placebo. Responders were defined as those who experienced an average reduction of 1.0 in the lower extremities on the Ashworth Scale for spasticity. Responders received intrathecal baclofen via the SynchroMed System and were followed for up to 43 months. Fifty-one patients completed screening and 44 entered open-label trials. Lower-extremity spasticity decreased from an average baseline score of 3.64 to 1.90 at 39 months. A decrease in upper extremity spasticity was evidenced over the same study period. Forty-two patients reported adverse events. Most common reports were hypotonia, seizures (no new onset), somnolence, and nausea or vomiting. Fifty-nine percent of the patients experienced procedural or system-related events. Spasticity in patients with cerebral palsy can be treated effectively by continuous intrathecal baclofen. Adverse events, although common, were manageable.

Adolescent↗

Meningeal diverticula treated with ventriculoperitoneal shunt.

An infant with Chiari malformation developed respiratory distress due to progressive intrathoracic enlargement of multiple spinal meningeal diverticulae. Ventriculoperitoneal shunting of the associated hydrocephalus temporarily halted the expansion of the intrathoracic diverticulae and permanently relieved respiratory symptoms. The role of hydrostatic pressure in the enlargement of meningoceles and other spinal meningeal diverticulae is discussed. Ventriculoperitoneal shunting is suggested as a surgical alternative for the treatment of symptomatic meningoceles.

Arnold-Chiari Malformation↗

Isolated CNS involvement in Ewing's sarcoma.

Ewing's sarcoma, an uncommon malignant neoplasm of bone, represents about 10% of all the malignant primary bone tumors. The assumption that subclinical metastases are already present in patients with apparently localized tumor indicates the need for systemic therapy as an integral part of primary treatment. The usual sites of metastases are the lungs and skeletal system. Central nervous system (CNS) involvement is rare and is usually seen only in disseminated and fairly advanced disease. We report two patients in whom, after aggressive adjuvant chemotherapy, disease developed in the CNS without local recurrence. In one patient, meningeal involvement with malignant cells was identifiable in the cerebrospinal fluid; in the second patient, who presented with a space-occupying lesion, a diagnosis of arachnoid involvement was made histologically.

Adolescent↗

Subarachnoid hemorrhage in sickle cell disease.

Subarachnoid hemorrhage occurs in 1%-2% of patients with neurological complications from sickle cell disease. The authors report a case of subarachnoid hemorrhage in a 13-year-old black girl with sickle cell disease. Computed tomography of the brain demonstrated diffuse subarachnoid hemorrhage in the right sylvian fissure and the right frontoparietal cortical region. Angiography revealed no evidence of aneurysm but multiple stenosis and/or occlusions of the distal branches of the anterior and middle cerebral arteries bilaterally. Rupture of leptomeningeal collateral vessels is a possible cause of subarachnoid hemorrhage in our patient.

Adolescent↗

Fetal hydrocephalus: a case of ventricular-amniotic shunting.

The case we have reported describes the management of fetal hydrocephalus using an intrauterine ventricular-amniotic shunt, which unfortunately was not recovered after delivery. To prevent this potential complication, we suggest such shunts be made radiopaque. The encouraging results in this case are attributed to the efforts of an interdisciplinary team and suggest that vascular accidents may well prove to be amenable to treatment by this new procedure.

Adult↗

Direct fetal therapy: the multidisciplinary team concept.

A multidisciplinary medical and surgical team is essential to direct fetal therapy. This group of skilled specialists is usually available only in university hospitals. Through the use of recently developed technology and a high degree of skill, the team composed of nonphysician and physician members can detect, evaluate, and occasionally treat affected patients. Through research, education,aand communication, the team endeavors to meet the need for direct fetal therapy in life-threatening malformations.

Congenital Abnormalities↗

Peduncular hallucinations caused by brainstem compression.

Peduncular hallucinations are associated with intrinsic lesions of the midbrain. We report transient peduncular hallucinations due to extrinsic compression of the midbrain by a cystic craniopharyngioma. The peduncular hallucinations resolved promptly after drainage of the cyst.

Brain Diseases↗

Peliosis hepatis. Twelve cases associated with oral androgen therapy.

Peliosis hepatis was encountered in 12 patients treated with high-dose oxymetholone or fluoxymesterone therapy. In three cases liver failure was the primary cause of death. In one case, the diagnosis was established by biopsy, the androgen therapy was discontinued, and the lesion was absent at autopsy two years later. In eight cases peliosis hepatis was an incidental finding at postmortem examination. The clinical and anatomic features of these cases are described, and previously reported cases of peliosis are briefly reviewed. A hypothesis is offered to explain the association of this peculiar lesion with anabolic androgen therapy.

Adult↗

Brain abscess caused by Cladosporium trichoides (Bantianum): a case with paranasal sinus involvement.

Dematiacious fungi (ie, fungi with dark hyphae) are a rare and usually fatal cause of central nervous system infection. Cladosporium trichoides has been implicated most frequently. Documentation of extra-CNS involvement has been rare. Our patient had a brain abscess and paranasal sinus infection due to C trichoides. The organism was sensitive to less than or equal 2mug/ml of flucytosine. Although our patient did not receive flucytosine, it is possible that this drug, because of its excellent penetration into the cerebrospinal fluid, may be a useful therapeutic agent in cerebral cladosporiosis.

Brain Abscess↗

Intra- and extracerebral arachnoid cyst. An atypical roentgenographic presentation.

Arachnoid cysts present in a variety of ways, and the preoperative diagnosis usually requires a high degree of suspicion. Bony changes consistent with a chronic focal mass effect, a relative paucity of clinical findings compared to the size of the lesion, and an extracerebral mass with an intracerebral component suggest the possibility. We present a case in which there appears to be a large, deep intracerebral mass associated with an extracerebral expansion.

Adult↗

Oxymetholone treatment for sickle cell anemia.

Seven patients with sickle cell anemia were treated with oxymetholone for at least 2 mo. Markedly increased basal rates of hemolysis and erythropoiesis were confirmed. The urinary erythropoietin excretion was either normal or lower than expected for the red cell mass, and an expanded blood volume was due primarily to an increased plasma volume. After androgen therapy, six patients demonstrated more than a fivefold increase in urinary erythropoietin, with an increase in red cell mass ranging from 17%-75% above the control value. All showed a decline in serum iron level to the 25-75 mug/100 ml range within 4 wk after the start of therapy. Less marked changes followed lower oxymetholone doses. Reversible hepatic toxicity, with a serum bilirubin concentration exceeding 50 mg/100 ml, occurred in one patient. Androgenic hormone therapy may be useful for selected adult patients with sickle cell disease when severe anemia contributes to disease morbidity.

Adolescent↗

Desmoplastic infantile ganglioglioma.

We present the clinical, anatomic, and laboratory findings in a 4-month-old child with desmosplastic infantile ganglioglioma. Microtubule-associated protein-2 (AP18) and neuron-specific B-tubulin (TUJ-1) were more sensitive in detecting immature neural elements than synaptophysin. Despite the immature neuroblastic component, focal intermediate proliferation indices, microinvasion, presence of secondary features (extension into Virchow Robin spaces, perineuronal satellitosis), and subtotal resection, the child has done well, with striking improvement of the magnetic resonance imaging (MRI) image, head size improvement, no tumor recurrence, and minimal neurological deficits.

Brain Neoplasms↗

C1-C2 posterior soft-tissue injuries with neurologic impairment in children.

A retrospective study of the initial lateral cervical spine radiographs (read as normal) of five multiply traumatized quadriplegic children showed a subtle widening between the spinous processes of C1 and C2. To verify the accuracy of this widening, five distances, two calculations, and four angles were defined and measured in the pathologic group and in a control group of 12 normal children. There was a significant difference (p less than 0.05) between the two groups. A widening of greater than 10 mm between the spinous processes of C1-C2 on a neutral lateral radiograph should alert the physician to an underlying spinal cord injury. The other measurements could then confine the pathology at that level.

Cervical Vertebrae↗