ABSTRACT
Langerhans cell histiocytosis (LCH) with multiple organ involvement is a rare disorder in adults. Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisensory loss and short-lasting episodes of an alien left hand due to lesions of the internal capsule and the right thalamus, extending into the mesencephalon associated with extensive surrounding edema, without pituitary involvement. The neuroradiological image suggested glioblastoma multiforme. Brain biopsy revealed inflammatory tissue and "pseudotumoral" multiple sclerosis was suspected. Biopsy of concomitant lung and bone lesions disclosed Langerhans cell histiocytosis. The treatment with pulsed steroids in association with mycophenolate mofetil led to a sustained, clinical neurological remission.
Subject(s)
Brain Diseases/diagnosis , Histiocytosis, Langerhans-Cell/diagnosis , Age of Onset , Alien Limb Phenomenon/etiology , Biopsy , Bone and Bones/pathology , Brain/pathology , Brain Diseases/complications , Brain Diseases/drug therapy , Brain Neoplasms/diagnosis , Cerebellar Ataxia/etiology , Dexamethasone/administration & dosage , Dexamethasone/therapeutic use , Diagnosis, Differential , Drug Therapy, Combination , Female , Glioblastoma/diagnosis , Histiocytosis, Langerhans-Cell/complications , Histiocytosis, Langerhans-Cell/drug therapy , Histiocytosis, Langerhans-Cell/epidemiology , Humans , Lung/pathology , Magnetic Resonance Imaging , Middle Aged , Multiple Sclerosis/diagnosis , Mycophenolic Acid/administration & dosage , Mycophenolic Acid/analogs & derivatives , Mycophenolic Acid/therapeutic use , Paresis/etiologyABSTRACT
In this study, a report has been made of 19 cases of severe encephalopathy in patients with renal impairment who were treated during the last three years for various infections with cefepime, a new parenteral cephalosporin antibiotic. All patients (aged 57 to 91 years) presented a prolonged confusional state associated with diffuse rhythmic non-reactive triphasic sharp waves on the EEG. All the electroclinical symptomatology disappeared within 24-48 hours after discontinuation of drug administration. A clear relation was found between encephalopathy and cefepime intake. These observations underline the fact that the cefepime dosage should be reduced in renally impaired patients.
