Yitzchak Frank

Topical Review: Hypertensive Encephalopathy, Reversible Occipitoparietal Encephalopathy, or Reversible Posterior Leukoencephalopathy: Three Names for an Old Syndrome

Children with hypertension, seizures, lethargy, encephalopathy, headache, and occipital blindness are reviewed. After undergoing antihypertensive therapy, most children improve. Some patients have a similar syndrome associated with chemotherapy, transplantation, transfusion, or human immunodeficiency virus-1 (HIV-1) infection. These latter children can develop symptoms with only minimal or no discernible elevations in blood pressure and improve, in the case of cancer-associated encephalopathy, after discontinuing chemotherapy. The reported children with this distinctive clinical condition are compared to adults with reversible posterior leukoencephalopathy syndrome. Since both gray and white matter are involved, we had suggested previously that the name be changed to (reversible) occipitoparietal encephalopathy syndrome. However, reversible posterior leukoencephalopathy has been used in the adult population and probably should be employed in children for the sake of uniformity, since both children and adults have the same clinical presentation and presumably a similar pathophysiology for the encephalopathy syndrome. The diagnosis is confirmed by reversible posterior abnormalities seen on T2-weighted brain magnetic resonance imaging, and by the presence of either headache, altered mental status, seizures, or visual disturbances. (J Child Neurol 1999;14:277-281).

Occipital-parietal encephalopathy: a new name for an old syndrome.

A boy presented with hypertension, seizures, lethargy, headache, and occipital blindness. He improved with antihypertensive therapy. Other reported children with a similar distinctive clinical condition are compared with adults with a syndrome termed reversible posterior leukoencephalopathy. Because both gray and white matter are involved, we suggest that the name be changed to occipital-parietal encephalopathy syndrome.

Multiple ischemic infarcts in a child with AIDS, varicella zoster infection, and cerebral vasculitis

A 4 1/2-year-old girl with acquired immunodeficiency syndrome and prolonged varicella zoster virus skin infection developed multiple ischemic strokes and radiologic and histopathologic evidence of central nervous system vasculitis. Typical features of acquired immunodeficiency syndrome encephalitis were not present and there was no evidence of vasculitis outside the nervous system. Central nervous system vasculitis probably resulted from varicella zoster virus infection that persisted because of immunodeficiency. This acquired immunodeficiency syndrome complication has only rarely been described in adults and to our knowledge has not been described in children.

Event-related potentials to an "oddball" auditory paradigm in children with learning disabilities with or without attention deficit hyperactivity disorder.

Event Related Potentials (ERP) were recorded during a two-tone discrimination task in 18 children with Learning Disabilities (LD) and 36 children with Learning Disabilities and Attention Deficit Hyperactivity Disorder (LD-ADHD). Twenty-seven normal children served as a control group. P3 wave amplitude was significantly smaller in the LD and LD-ADHD groups compared with normal children. There was no difference between the LD and LD-ADHD groups. It is suggested that the smaller P3 amplitude in children with LD-ADHD syndromes reflects cognitive and processing difficulties, which frequently coexist with ADHD in these children and is not specifically related to an attention deficit.

CHILDHOOD AIDS, VARICELLA ZOSTER, AND CEREBRAL VASCULOPATHY

Case Report An 8-year-old girl presented to North Shore University Hospital with new onset of left third nerve palsy and a macular rash on her left cheek and around the left eye. She had a history of vertically acquired human immunodeficiency virus (HI~-1 infection, met clinical criteria for AIDS, and was treated with zidovudine and 2;3’-dideoxyinosine. Investigation revealed no evidence of toxoplasmosis, cytomegalovirus, Mycobacterium avium intracellulare,

Malignant rhabdoid tumor of the brain and kidney in a child: Clinical and pathologic features

Malignant rhabdoid tumor (MRT) is most frequently found in the kidney, but can occur in other tissues including the brain. The simultaneous appearance of MRT in the brain and kidney has rarely been described. We report the first fully described case of simultaneous appearance of MRT in the kidney and cerebellum of an 8-month-old boy. Cytoplasmic inclusion-like masses, representing aggregates of intermediate filaments, positively stained by Vimentin and by epithelial membrane antigen, were abundant in the kidney tumor but rare in the cerebellar tumor, suggesting that this often-described characteristic of kidney MRT can be an infrequently observed feature of brain MRT, which consequently may be thought to represent another type of tumor.

Brainstem auditory evoked responses in infants and children with AIDS

Brainstem auditory evoked responses were measured in 16 infants and children with acquired immunodeficiency syndrome (AIDS) and in 9 normal infants and children. Two stimulation rates were used: a conventional rate of 10 Hz and a high rate of 50 Hz. Latencies of waves III, IV, and V on the left were significantly longer in the AIDS group when a stimulation rate of 10 Hz was used. With a higher stimulation rate of 50 Hz, significant differences between the two groups occurred in the latencies of waves I, III, and V bilaterally, but there were no significant differences in the interpeak latencies. A measure of the differential effect of the increasing stimulus rate on the two groups was significant for wave I latency and for I-III and I-V interpeak latencies on the left, revealing that increasing stimulation rate prolongs these measures more in the AIDS group. Increased brainstem auditory evoked response stimulation rate may unmask abnormalities in infants and children with AIDS that are not observed when the lower stimulation rate is used.