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Biomedical subjects

M I Steinlin

Publications and source records attributed to M I Steinlin.

3 recordsLinked to original sources

Late intrauterine Cytomegalovirus infection: clinical and neuroimaging findings.

Fetal Cytomegalovirus (CMV) infection in early pregnancy usually results in severe neurological handicap and sensorineural hearing loss with typical neuroradiological findings of calcification, migrational anomalies, disturbed myelination, and cerebellar hypoplasia. Infections acquired in late pregnancy have less prominent signs, such as microcephaly, hearing deficits, and minor neurological handicap. We report 7 children who presented with a similar clinical complex of signs: microcephaly, sensorineural hearing impairment, behavior problems with hyperactivity, reduced apprehension for pain in 5 of the 7, ataxia in 3, and hypotonia with clumsiness in 3 others. All manifested mild to severe developmental problems. Cranial CT revealed calcification in 4 of 6 patients. MRI in all 7 children showed patchy to confluent nonprogressive dysmyelination. Only 2 children had acute neonatal signs of congenital CMV infection. We assume that these children acquired CMV infection in the third trimester of gestation, leading to microcephaly, hearing loss, and neurological and developmental problems with typical neuroradiological signs.

Child↗

Eye problems in children with multiple sclerosis.

In a retrospective review, the eye symptoms of 17 children (mean age: 13 1/2 years) who had definite multiple sclerosis (Poser's criteria) and 15 who had probable multiple sclerosis over the last 18 years were evaluated. Follow-up varied from 3 weeks to 6 years. Of 94% of children (16 of 17) with ophthalmologic symptoms, 47% (8 of 17) presented with an initial disturbance of vision. Twelve children had optic neuritis, 1 progressive uveitis, and 4 brainstem symptoms (i.e., VIth nerve palsy, 1 1/2 syndrome, internuclear ophthalmoplegia). Four children had cerebellar signs (nystagmus, saccadic pursuit). In 4 children, clinical localization was less specific. Recovery was generally good in most of the children; cerebellar problems were most persistent. Multimodal potentials were more helpful for investigation of optic neuritis and cerebellar lesions than for brainstem lesions. In the cohort of probable multiple sclerosis of 15 children, 11 had eye symptoms (5 with neuromyelitis optica, 4 optic neuritis, 1 internuclear ophthalmoplegia, and 1 cerebellar symptoms). Ophthalmologic symptoms are slightly more frequent in children with multiple sclerosis than in adults and should be specifically investigated to establish the diagnosis.

Adolescent↗

Neurologic manifestations of pediatric systemic lupus erythematosus.

Central nervous system involvement is a common but rarely reviewed feature of pediatric systemic lupus erythematosus (SLE). We retrospectively reviewed the charts of 91 patients with pediatric SLE and using a standardized data abstraction form documented 40 patients with central nervous system (CNS-SLE) involvement. The mean age of onset of SLE was 13.3 years. In 19 patients the CNS manifestation was a presenting symptom, in 12 patients CNS involvement was present within the first year of diagnosis, and in 9 patients it took up to 7 years for CNS disease to become evident. Nineteen children (48%) manifested neuropsychiatric SLE, which included depression, concentration or memory problems, and frank psychosis. Seizures were present in 8 patients (20%), 6 had cerebral ischemic events (15%), 1 had chorea (3%), 2 had papilledema (5%), and 2 patients had a peripheral neuropathy (5%). Nine patients (22%) had severe headache consistent with lupus headache. Seven children had more than one CNS manifestation. In the investigation of CNS-SLE, computed tomography and/or magnetic resonance imaging scans were helpful in patients with focal ischemic lesions and venous sinus thrombosis. Electroencephalography was abnormal only in 33% of patients with seizure disorders and rarely helpful in patients with diffuse neuropsychiatric symptoms. Single-photon emission computed tomography scans were abnormal in most patients with neuropsychiatric SLE, especially in those with frank psychosis. The lupus anticoagulant was present in the patient with chorea and was frequently present in patients with cerebral vascular events. Long-term outcome was good: only 1 child died of cerebral hemorrhagic infarction and 3 others had significant persistent CNS deficits. The majority of patients (90%) had excellent recovery from CNS-SLE.

Adolescent↗