Biomedical subjects

Lawrence P Richer

Publications and source records attributed to Lawrence P Richer.

4 recordsLinked to original sources

PubMed2006

Tuberous sclerosis with open lipped schizencephaly.

Tuberous sclerosis is a multisystem disorder that is transmitted in an autosomal dominant fashion. It affects approximately 1 in 10,000 live births. A growing body of evidence implicates a defect of cell maturation and migration in the pathogenesis of many of the lesions in tuberous sclerosis affecting the central nervous system. There have been numerous case reports of various abnormalities of neuronal migration associated with tuberous sclerosis. To our knowledge, there has only been one case of schizencephaly reported in a patient with tuberous sclerosis. The present report describes a male with tuberous sclerosis and an extensive open lipped schizencephalic cleft affecting his right frontoparietal lobe. This case supports the hypothesis that the cerebral lesions in tuberous sclerosis are secondary to a defect in neuronal maturation and migration.

Abnormalities, Multiple↗

PubMed2005

Neuroimaging features of acute disseminated encephalomyelitis in childhood.

Acute and disseminated demyelination of the central nervous system in children may have many causes. This study reports a retrospective cohort of 10 consecutive pediatric cases (5 to 17 years; mean [S.D.] 12.1 [4.3] years) with a presenting diagnosis of acute disseminated encephalomyelitis and the clinical and radiologic follow-up. The evolution of magnetic resonance imaging abnormalities was determined from serial studies in nine patients with a mean (S.D.) follow-up of 334.2 (312.8) days. Resolution of magnetic resonance imaging T(2) prolongation (i.e., demyelination) within 6 months of presentation was associated most commonly with a final clinical diagnosis of acute disseminated encephalomyelitis (including the multiphasic form), but failed to meet statistical significance (Fisher's exact test; P = 0.083). Incomplete resolution of the magnetic resonance imaging signal abnormalities yielded a statistically significant association (Fisher's exact test; P = 0.048) with an abnormal neurologic outcome. In summary, we conclude that early resolution of magnetic resonance imaging signal abnormalities or recurrent demyelination within 6 months of an acute and disseminated demyelinating event suggests the diagnosis of acute disseminated encephalomyelitis in children, although this association failed to meet statistical significance. However, a statistically significant association between complete resolution of magnetic resonance imaging signal abnormalities and a normal neurologic outcome was observed.

Adolescent↗

PubMed2002

Epileptiform abnormalities in children with attention-deficit-hyperactivity disorder.

The proportion of children with attention-deficit-hyperactivity disorder and epileptiform abnormalities is compared with an historic control group of normal school-aged children. The medical records of 655 children 5-16 years of age referred to a single pediatric neurologist (M.S.) from January 1991 to December 1999 with school problems, behavior problems, or hyperactivity were retrospectively reviewed. Clinical criteria for attention-deficit-hyperactivity disorder were satisfied in 476 of these children. An electroencephalogram was obtained from 347 patients and coded as epileptiform in 6.1 +/- 1.3%, which is significantly higher (chi-square test, P < 0.025) than the prevalence rate of 3.5 +/- 0.6% observed in a study of 3,726 normal school-aged children. The epileptiform abnormality was present only with activation procedures in six of our patients (hyperventilation [n = 2] and photic stimulation [n = 4]). Only three of the 21 children with epileptiform abnormalities developed a seizure disorder in our cohort. We conclude that the prevalence rate of epileptiform abnormalities is greater in children with attention-deficit-hyperactivity disorder compared with that observed in normal school-aged children when hyperventilation and photic stimulation are used. However, the clinical utility of routine electroencephalography in the diagnosis of a comorbid seizure disorder in children with attention-deficit-hyperactivity disorder is limited.

Attention Deficit Disorder with Hyperactivity↗

PubMed2002

Pediatric meralgia paresthetica.

Meralgia paresthetica is a focal peripheral neuropathy involving the lateral femoral cutaneous nerve and is rarely observed in pediatric practice. Previous reports have highlighted its occurrence within the context of a regional bony malignancy. We present here three patients less than 18 years of age with idiopathic meralgia paresthetica.

Adolescent↗