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Acta neurologica Belgica. 04/2013;
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Journal of Paediatrics and Child Health 08/2012; 48(8):709, 714. · 1.28 Impact Factor
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Neurology 07/2012; 79(2):e7-9. · 8.31 Impact Factor
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Journal of child neurology 04/2012; · 1.59 Impact Factor
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ABSTRACT: Cerebral vasculopathy is an important but underrecognized complication of neurofibromatosis type 1. Over a 10-year period, we retrospectively assessed the prevalence, clinical manifestations, management, and outcome of cerebral vasculopathy in children with neurofibromatosis type 1. Magnetic resonance imaging (MRI) of the brain was performed on 78% of the patients (312/398) of which 46% (143/312) had magnetic resonance angiography of the intracranial arteries; 4.8% (15/312) had cerebral vasculopathy. Approximately half were asymptomatic at presentation; none had neurologic deficits. Cerebral vasculopathy included moyamoya changes (7) and stenosis/occlusion of major intracranial arteries (8). On follow-up (mean 4 years), 2 patients developed radiologic progression; 1 was treated with aspirin alone, whereas another underwent revascularization surgery. Although cerebral vasculopathy in neurofibromatosis type 1 may be asymptomatic at presentation, there may be radiologic and clinical progression leading to morbidity and mortality. Magnetic resonance angiography should be considered with brain MRI for early detection and timely intervention of cerebral vasculopathy.
Journal of child neurology 04/2012; · 1.59 Impact Factor
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Journal of Paediatrics and Child Health 02/2012; 48(2):179, 187. · 1.28 Impact Factor
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ABSTRACT: Hirayama disease has been mainly reported from Asia; only a few cases are from the Western hemisphere, particularly North America. This is a retrospective chart review of patients < 18 years, diagnosed with Hirayama disease from a single center over 10 years. We diagnosed 6 children (4 boys), 15.1 ± 1.2 years of age. Symptom onset was 3 months to 3 years before presentation. All had unilateral or bilateral asymmetric distal upper extremity weakness without objective sensory loss. Oblique amyotrophy and cold paresis were noted in 5. On electromyography, acute-on-chronic denervation was most frequently noted in cervical-8 (C8) and thoracic-1 (T1) myotomes followed by cervical-7 (C7) myotome in both upper limbs, sparing C5-C6 myotomes. Cervical magnetic resonance imaging (MRI) was abnormal in 3. Symptoms progressed over a mean of 16.5 months. Treatment consisted of placement of cervical collar. Heightened awareness of this entity among pediatric neurologists in North America will lead to early diagnosis and intervention, avoiding unnecessary investigations.
Journal of child neurology 12/2011; 26(12):1542-7. · 1.59 Impact Factor
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Neurology 11/2011; 77(19):e114. · 8.31 Impact Factor
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ABSTRACT: The authors report an 8-year-old girl with refractory status epilepticus due to hypertensive encephalopathy, secondary to end-stage renal disease. Brain magnetic resonance imaging (MRI) in the acute phase showed striking hyperintensities in the brain stem and medial thalamus along with subtle cortical lesions. After successful control of hypertensive crisis and status epilepticus, the patient recovered to her baseline. Near total resolution of the lesions was noted on follow-up imaging performed 9 days later. Predominant brainstem involvement as a feature of posterior reversible encephalopathy syndrome due to hypertensive crisis is extremely rare in children and has not been well documented.
Journal of child neurology 04/2011; 26(8):1033-5. · 1.59 Impact Factor
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ABSTRACT: Of patients admitted to hospital with sinusitis, about 3% have an intracranial complication. We describe the clinical features, laboratory data, imaging findings, and outcomes of pediatric patients with intracranial complications of sinusitis.
A retrospective chart review of all pediatric patients < 21 years of age admitted for intracranial complications of sinusitis to a tertiary hospital over a 10-year period.
A total of 13 patients with a mean age of 13.3 ± 3.7 years presented with headache (92%), fever (85%), nausea/vomiting (62%), sinus tenderness (31%), and lethargy (23%). Physical examination findings included Pott puffy tumor (46%), orbital cellulitis (46%), altered level of consciousness (46%), new-onset seizure (31%), hemiparesis (23%), nuchal rigidity (23%), visual disturbance (23%), and slurred speech (15%). Computed tomography and magnetic resonance imaging demonstrated 16 instances of epidural and subdural empyema, and 1 brain abscess. One child had sagittal sinus thrombosis.
Our findings suggest that acute sinusitis in combination with severe intractable headache, varying degrees of altered level of consciousness, focal neurologic deficits, and/or signs of meningeal irritation should raise clinical suspicion for potential intracranial complications of sinusitis. These signs and symptoms should prompt early and aggressive evaluation and management, including neuroimaging studies, neurologic and otolaryngologic consultations, and intravenous antibiotics.
The Pediatric Infectious Disease Journal 03/2011; 30(3):222-6. · 3.58 Impact Factor
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ABSTRACT: The authors describe the demographics, clinical presentation, investigation, treatment, and outcomes of pediatric patients with Guillain-Barré syndrome. They identified 35 pediatric patients with Guillain-Barré syndrome presenting to a tertiary academic center over a 20-year period. The most common presenting symptoms were paresthesias (54%), weakness (49%), and myalgias (49%). Sensation was affected in 54% of patients, and hyporeflexia or areflexia was present in 94% of patients. Cranial nerve dysfunction (46%) and autonomic involvement (eg, changes in blood pressure, pulse, bowel/bladder control, or priapism; 46%) were also common. Autonomic dysfunction, cranial nerve involvement, and albuminocytological dissociation were significantly associated with a decreased time to nadir, the point when symptoms peaked (P = .015, .007, and .005, respectively). Although not statistically significant, treatment with plasmapheresis had a better success rate than intravenous immunoglobulin. The authors' results will help to further delineate the clinical picture of Guillain-Barré syndrome in children and refine treatment strategies.
Journal of child neurology 12/2010; 25(12):1504-10. · 1.59 Impact Factor
