Biomedical subjects

W J Borkowski

Publications and source records attributed to W J Borkowski.

6 recordsLinked to original sources

PubMed1997

Obstructive sleep apnea in Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome (SJS) is a rare entity characterized by myotonia and skeletal abnormalities. Death and respiratory distress have previously been reported in newborns and young children with SJS. We describe a patient with SJS and snoring in whom polysomnography demonstrated obstructive sleep apnea and hypoxia. Although tonsillectomy with laser palatoplasty significantly widened the oropharyngeal introitus, obstructive sleep apnea persisted. Ultimate improvement occurred only after the institution of home therapy with bi-level positive airway pressure during the night. We also discuss the specific structural and neuromuscular features of SJS that may be responsible for upper airway obstruction.

Adult↗

PubMed1992

Effect of sleep deprivation on the EEG of learning-impaired children with absence seizures.

Learning-impaired children can present with a clinical history suggesting additional problems, including attention deficit disorder and absence seizures. Physicians use clinical judgment in diagnosing the former, but rely heavily on the electroencephalogram (EEG) to diagnose the latter. Most clinicians are reluctant to treat patients with anticonvulsants without seeing 3 Hz spike-and-wave discharges in an EEG. We studied 21 learning-impaired and 7 control children, ages 5 through 12 years, with normal baseline EEGs. All had an additional EEG recorded after 24 hour sleep deprivation. Nine learning-impaired children had a history compatible with absence seizures; 8 of them showed spike-and-wave discharges after sleep deprivation. Of the 12 learning-impaired children with no history of absence seizures, 2 showed spike-and-wave discharges after sleep deprivation. These results are significant (p less than 0.001). Of the 7 controls, none had epileptiform activity following sleep deprivation. Our results show that routine EEGs may be falsely negative in patients with absence seizures, and that sleep deprivation is a potent activator.

Cerebral Cortex↗

PubMed1989

Pseudallescheria boydii brain abscess: association with near-drowning and efficacy of high-dose, prolonged miconazole therapy in patients with multiple abscesses.

Brain abscess caused by Pseudallescheria boydii is a highly lethal infection, usually seen in immunosuppressed patients. Five patients with P. boydii brain abscesses are described. Four of these patients acquired their infection after near-drowning; 1 patient developed an abscess after penetrating head trauma. Two patients survived their infections, which included involvement of other body sites (lung, eye, bone) as well as multiple undrained brain abscesses, after prolonged courses of high-dose parenteral miconazole (80-90 mg/kg/d). Progressive increases in miconazole dosage during the treatment periods were required to produce serum levels above the minimum inhibitory concentrations of the fungal isolates.

Adult↗

PubMed1981

Valproic acid and secondary hyperammonemia.

An 11-year-old girl with complex seizures was started on valproic acid (VPA) in addition to clonazepam and ethosuximide. Shortly thereafter, she developed marked hyperammonemia that was worsened by a protein load. The hyperammonemia improved somewhat when protein was not given, and it resolved on discontinuation of the valproic acid. No associated changes in serum transaminases or bilirubin were observed. Isolated hyperammonemia may occur soon after VPA ingestion and appears to be a relatively infrequent, reversible side effect. The mechanism of hyperammonemia probably differs from other manifestations of hepatotoxicity, such as elevated transaminases or frank hepatic failure.

Ammonia↗

PubMed1980

Progressive multifocal leukoencephalopathy and malignant lymphoma of the brain in a patient with immunosuppressive therapy.

Progressive multifocal leukoencephalopathy and malignant lymphoma of the brain were noted at postmortem examination in a 68-year-old white woman who was treated with immunosuppressive agents after renal transplantation. The two diseases are not uncommon in patients with immunodeficiency, but their occurrence in the same patient is extremely rare. This association suggests the oncogenicity of papova viruses in man. However, no papovavirus was demonstrated in the tumor by electron microscopy and immunohistochemical staining. The immunohistochemical staining of routine histology sections for the common antigen of polyomaviruses by the peroxidase anti-peroxidase technique is shown to be simple and specific for the detection of polyomaviruses in the demyelinated areas of progressive multifocal leukoencephalopathy.

Aged↗

PubMedDate not supplied

Cerebrospinal fluid parameters and auditory brainstem responses following meningitis.

Auditory brainstem responses were measured in 94 children under 24 months of age immediately following treatment for bacterial meningitis. Evidence of peripheral hearing loss (thresholds of 30 dB HLn or greater) was found in 47% of the patients. In addition, 9% had prolonged interwave latencies, indicating the possible presence of retrocochlear pathology. Other clinical data were examined as well. CSF glucose concentration correlated with both the presence and magnitude of hearing loss (as measured by auditory brainstem responses). Magnitude of hearing loss also was associated with the presence of seizures. Although all children recovering from meningitis should be assessed for hearing loss, those who have had low CSF glucose concentrations and seizures appear to be at high risk.

Blood Glucose↗