Sarit Ravid

Long-term motor and cognitive outcome of acute encephalitis.

OBJECTIVES: To examine the long-term motor and neurocognitive outcome of children with acute encephalitis and to look at possible prognostic factors. METHODS: Children who were treated for acute encephalitis in 2000–2010 were reevaluated. All children and their parents were interviewed by using structured questionnaires, and the children underwent full neurologic examinations, along with comprehensive neurocognitive, attention, and behavioral assessments. RESULTS: Of the 47 children enrolled, 1 died and 29 had neurologic sequelae, including motor impairment, mental retardation, epilepsy, and attention and learning disorders. Children with encephalitis had a significantly higher prevalence of attention-deficit/hyperactivity disorder (50%) and learning disabilities (20%) compared with the reported rate (5%–10%) in the general population of Israel (P < .05) and lower IQ scores. Lower intelligence scores and significantly impaired attention and learning were found even in children who were considered fully recovered at the time of discharge. Risk factors for long-term severe neurologic sequelae were focal signs in the neurologic examination and abnormal neuroimaging on admission, confirmed infectious cause, and long hospital stay. CONCLUSIONS: Encephalitis in children may be associated with significant long-term neurologic sequelae. Significant cognitive impairment, attention-deficit/hyperactivity disorder, and learning disabilities are common, and even children who were considered fully recovered at discharge may be significantly affected. Neuropsychological testing should be recommended for survivors of childhood encephalitis.

A novel homozygous splice site mutation in NALCN identified in siblings with cachexia, strabismus, severe intellectual disability, epilepsy and abnormal respiratory rhythm.

We studied three siblings, born to consanguineous parents who presented with severe intellectual disability, cachexia, strabismus, seizures and episodes of abnormal respiratory rhythm. Whole exome sequencing led to identification of a novel homozygous splice site mutation, IVS29-1Gxa0>xa0A in the NALCN gene, that resulted in aberrant transcript in the patients. NALCN encodes a voltage-independent cation channel, involved in regulation of neuronal excitability. Three homozygous mutations in the NALCN gene were previously identified in only eight patients with severe hypotonia, speech impairment, cognitive delay, constipation and Infantile-Neuroaxonal-dystrophy- like symptoms. Our patients broaden the clinical spectrum associated with recessive mutations in NALCN, featuring also disrupted respiratory rhythm mimicking homozygous Nalcn knockout mice.

Reactivation of Varicella Presenting as Pseudotumor Cerebri: Three Cases and a Review of the Literature

The possible association of pseudotumor cerebri and varicella infection was previously mentioned in a few case reports. In those cases, the history and clinical features of active varicella were obvious, and signs were directly related to the varicella infection. We describe three immunocompetent children with pseudotumor cerebri as the only manifestation of Varicella zoster virus reactivation, with a review of the literature. We suggest considering Varicella zoster virus in children with pseudotumor cerebri, even in the absence of a history of recent varicella infection.

Long-term motor, cognitive and behavioral outcome of acute disseminated encephalomyelitis

OBJECTIVEnThe purpose of this study was to evaluate the long-term motor and neurocognitive outcome of children with acute disseminated encephalomyelitis and to identify prognostic risk factors.nnnMETHODSnThe study included 43 children who were hospitalized due to acute disseminated encephalomyelitis during the years 2002-2012. The children underwent full neurological examinations, along with comprehensive neurocognitive and behavioral assessments.nnnRESULTSnTwenty-six (61%) children had different degrees of neurological sequelae after a mean follow-up of 5.5 ± 3.5 years. The most common residual impairment included attention-deficit hyperactivity disorder (44%), behavioral problems (32%), and learning disabilities (21%). Five (12%) children had a full-scale IQ of 70 or less, compared to 2.2% in the general population.nnnCONCLUSIONSnNeurocognitive sequelae were found even in children who were considered as fully recovered at the time of discharge. Risk factors for severe neurological sequelae were older age at diagnosis and male gender. We suggest neuropsychological testing and long-term follow-up for all children with acute disseminated encephalomyelitis, even in the absence of neurological deficits at discharge.

Viral-Induced Intracranial Hypertension Mimicking Pseudotumor Cerebri

BACKGROUNDnPseudotumor cerebri or idiopathic intracranial hypertension is characterized by normal spinal fluid composition and increased intracranial pressure in the absence of a space-occupying lesion.nnnMETHODSnThis study describes a subgroup of 10 patients with the same typical presenting symptoms (headache, vomiting, and papilledema) but without nuchal rigidity, meningeal signs, or change in mental status. Patients had normal neuroimaging studies and intracranial hypertension but also pleocytosis in the cerebrospinal fluid, suggesting central nervous system infection. From the results it can be hypothesized that those children represent a unique subgroup of viral-induced intracranial hypertension when comparing their risk factors, clinical course, treatment, and outcome with 58 patients who had idiopathic intracranial hypertension.nnnRESULTSnAll patients with viral-induced intracranial hypertension presented with papilledema but none had reduced visual acuity or abnormal visual fields, compared with 20.7% of patients who had idiopathic intracranial hypertension. They also responded better to treatment with acetazolamide, needed a shorter duration of treatment (7.7 ± 2.6 months vs 12.2 ± 6.3 months, Pxa0= 0.03), and had no recurrences.nnnCONCLUSIONSnThe results suggest that children who fulfill the typical presenting signs and symptoms and all diagnostic criteria for pseudotumor cerebri other than the normal cerebrospinal fluid component may represent a unique subgroup of viral-induced intracranial hypertension and should be managed accordingly. The overall prognosis is excellent.

Visual Outcome and Recurrence Rate in Children With Idiopathic Intracranial Hypertension

The purpose of this retrospective study was to evaluate the visual outcome and recurrence rate of idiopathic intracranial hypertension in children. The study included 68 patients who were diagnosed with idiopathic intracranial hypertension according to the modified Dandy criteria. Permanent visual impairment was rare. Three percent remained with mild visual impairment, 4% with minimal visual field defects, and only 1 patient had severe visual impairment. However, 26% had either a prolonged course of disease or a recurring condition. Higher cerebrospinal fluid opening pressure was the only clinical predictor at presentation (P = .04). Recurrence rate was 18%, and in most cases, the second episode occurred during the first year after remission. There was no significant difference between the group of patients with only 1 episode and the group of patients with more than 1 episode. We suggest long-term follow-up after remission, for at least a year, for all children with idiopathic intracranial hypertension.

Legionella pneumophila Pneumonia in Two Infants Treated with Adrenocorticotropic Hormone

Patients with infantile spasms, an intractable epileptic disorder, often are treated with adrenocorticotropic hormone. Legionella pneumophila is a rare cause of pneumonia in children. We describe 2 infants with Legionella pneumonia whose infection occurred within 1 month after starting adrenocorticotropic hormone.

Clival Syndrome Secondary to Anaerobic Mastoiditis in A 2-Year-Old Child.

Complications of acute mastoiditis can occur in about 10-20% of cases. Clival syndrome is a rare complication of mastoiditis, involving the 6th and 12th cranial nerves. We describe a case of a child with mastoiditis and presumed Lemierre syndrome complicated by clival syndrome.

PP15.12 – 2382: Chronic daily headache in children: Association with obesity, medication overuse and disability

Objective To examine the association between obesity, medication overuse and chronic daily headaches (CDH) in children, and the relation to headache type and disability. Methods The authors retrospectively evaluated 108 children with chronic daily headaches from their Pediatric Neurology Clinic. Data regarding age, gender, headache type, duration, disability and use of medication, along with height and weight were collected. Body mass index (BMI) was calculated, and percentiles were determined for age and sex. Headache type, disability and use of medications were compared between normal weight, at risk for overweight and overweight children. Results A higher prevalence (39. %) of obesity was found in the whole study group compared to the general population. The diagnosis of transformed migraine, but not of chronic tension type headache, was significantly associated with being at risk for overweight (OR=2.39, 95% CI: 1.2–4.69, P=0.01) or overweight (OR=2.3, 95% CI: 1.3–5.61, P=0.04). Regardless of headache type, a high BMI percentile was associated with increased headache disability and overuse of medications, but not with duration of attacks. Conclusion Obesity and CDH in children are associated. Although obesity seems to be a risk factor for transformed migraine more than for chronic tension type headache, it is associated with increased headache disability and medication overuse regardless of headache type.