Mahendranath Moharir

Anticoagulants in pediatric cerebral sinovenous thrombosis: a safety and outcome study.

Clinical trials are lacking in pediatric cerebral sinovenous thrombosis (CSVT). Neonates and children increasingly receive anticoagulant therapy (ACT) based on adult studies. Safety data for ACT in pediatric CSVT are scant and urgently needed. The objective was to assess the safety and outcome of ACT in pediatric CSVT.

Diagnostic yield of genetic testing in epileptic encephalopathy in childhood

Epilepsy is a common neurologic disorder of childhood. To determine the genetic diagnostic yield in epileptic encephalopathy, we performed a retrospective cohort study in a single epilepsy genetics clinic.

Anti–NMDA Receptor Encephalitis

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe but potentially reversible neurologic disorder that is clinically recognizable in children and adolescents. Prompt diagnosis and treatment are essential to facilitate recovery. Treatment consists of corticosteroids, intravenous immunoglobulin, or plasma exchange as first-line therapy followed by cyclophosphamide or rituximab, if necessary, as second-line immunotherapy. Patients with tumor-associated encephalitis benefit from tumor resection. More than 75% of patients make a substantial recovery, which occurs in the reverse order of symptom presentation associated with a decline in antibody titers.

A prospective outcome study of neonatal cerebral sinovenous thrombosis.

Neonatal cerebral sinovenous thrombosis is a frequent contributor to neonatal mortality and morbidity. Treatment is controversial, and reported clinical outcomes vary widely. Newborns with radiologically confirmed neonatal cerebral sinovenous thrombosis from 1992 to 2009 were prospectively followed in our Children’s Stroke Clinic for standardized outcomes, including the Pediatric Stroke Outcome Measure. Outcomes were available in 90 of 104 (87%) neonates. Early outcomes included cerebral sinovenous thrombosis-associated death (5) and thrombus propagation (15 [6 associated with new venous infarcts]). Lack of anticoagulation predicted propagation (RR = 13; P = .0007). Complete thrombus recanalization occurred in 90% by 3 months. Late outcomes (median, 2.5 years) were epilepsy (15) and neurological disability (50), which included moderate-severe language (43), sensorimotor (38), and cognitive/behavioral (24) deficits. Overall, 61% had poor outcome (death/any deficit). Concurrent neurological comorbidity at diagnosis (odds ratio = 2.8; P = .029) predicted poor outcome. Clinical trials are urgently needed to establish more effective treatment strategies.

Long-Term Outcomes of Pediatric Ischemic Stroke in Adulthood:

This population-based study assesses the long-term impact of childhood stroke on function and independence in young adults. We undertook a cross-sectional outcome study of patients with arterial ischemic stroke and cerebral sinovenous thrombosis, beyond 18 years of age. We studied 26 patients; 21 arterial stroke, 5 cerebral sinovenous thrombosis, with 16 females. Mean age at assessment was 21.5 years, and mean follow-up time was 10.8 years. According to the modified Rankin Scale, final outcomes were 37% normal, 42% mild, 8% moderate, and 15% severe deficits. Risk factors for abnormal functional outcome included arterial ischemic stroke, presence of arteriopathy, and 1-year poststroke Pediatric Stroke Outcome Measure score ≥ 2 (P < .05). Most (77-84%) were independent in driving, relationships, and employment. Functional status at 1 year poststroke strongly predicts long-term outcome. Mental illness in one-quarter of young adults surviving childhood stroke represents an important direction for research.

Intellectual ability and executive function in pediatric moyamoya vasculopathy

Aim  Moyamoya vasculopathy is characterized by progressive stenosis of the major arteries of the Circle of Willis, resulting in compromised cerebral blood flow and increased risk of stroke. The objectives of the current study were to examine intellectual and executive functioning of children with moyamoya and to evaluate the impact of moyamoya type, stroke (clinical or silent), vasculopathy laterality, and disease duration on neurocognitive abilities.

Childhood central nervous system vasculitis.

Inflammatory brain diseases in childhood are underrecognized and lead to devastating yet potentially reversible deficits. New-onset neurologic or psychiatric deficits in previously healthy children mandate an evaluation for an underlying inflammatory brain disease. Distinct disease entities, such as central nervous system (CNS) vasculitis, are now being increasingly reported in children. Clinical symptoms, initial laboratory test, and neuroimaging studies help to differentiate between different causes; however, more invasive tests, such as lumbar puncture, conventional angiography, and/or brain biopsy, are usually necessary before the start of treatment. This article focuses on childhood CNS vasculitis.

Basilar artery strokes in children: good outcomes with conservative medical treatment

To describe outcomes and outcome predictors in childhood basilar artery stroke (BAS).

Association between radiographic Wallerian degeneration and neuropathological changes post childhood stroke.

Aim  Wallerian degeneration is a radiological finding thought to reflect corticospinal tract degeneration. This finding on magnetic resonance imaging (MRI) is routinely used as a predictor of poor prognosis in childhood stroke. However, its validity has never been established. Our objective was to correlate Wallerian degeneration seen on MRI with histopathology.

Infantile Spasms as an Adverse Outcome of Neonatal Cortical Sinovenous Thrombosis

Cerebral sinovenous thrombosis is a rare but potentially serious condition often occurring in children with nonspecific presenting features. Much remains to be learned about the long-term outcome of infants with cerebral sinovenous thrombosis. We report a series of four patients taken from a prospective database of neonates with sinovenous thrombosis who subsequently developed infantile spasms, three with hypsarrythmia on electroencephalography and one with multiple independent spike foci. The first patient presented at 2 weeks of age with hypernatremia, dehydration, and seizures. He was found to have extensive thrombosis and hemorrhagic infarction of the right basal ganglia. The second patient presented at 5 weeks of life and was found to have sagittal sinus thrombosis with bilateral intracranial hemorrhage. The third patient presented with seizures on day 1 of life and was found to have venous thrombosis involving the torcular, extending into the sagittal sinus. The fourth patient presented at 3 weeks with lethargy and seizures. He was diagnosed with bacterial meningitis and also had extensive sinus thrombosis. All patients developed infantile spasms at ages 9, 7, 11, and 10 months, respectively. This is the first report in the English literature describing infantile spasms as a possible outcome of sinovenous thrombosis in early infancy. (J Child Neurol 2006;21:126—131; DOI 10.2310/7010.2006.00028).