Jaime Lin

TOXOCARIASIS OF THE CNS SIMULATING ACUTE DISSEMINATED ENCEPHALOMYELITIS

Toxocariasis is the infection of the human host with either Toxocara canis or Toxocara catis . Their definitive hosts are the domestic dog and cat, in which they live as adults within the lumen of the small intestine.1 Humans become infected after ingesting embryonated eggs from soil (geophagia, pica) or after exposure to dirty hands, raw vegetables, or larvae from undercooked giblets.2nnThe dominant clinical manifestations associated with toxocariasis are classified according to the organs affected. There are two main syndromes: visceral larvae migrans (VLM), which encompasses diseases associated with the major organs; and ocular larva migrans, in which pathologic effects on the host are restricted to the eye and the optic nerve.1,3–5 Clinical involvement of the CNS due to Toxocara species is rare and probably occurs as a result of hematogenous dissemination.4nnThis report describes a 2-year-old girl presenting neurotoxocariasis and an MRI pattern similar to acute disseminated encephalomyelitis (ADEM).nnA 2-year-old girl presented monthly episodes of fever and cough for approximately 1 year. Treatment during these episodes …

Leukoencephalopathy With Brainstem and Spinal Cord Involvement and Normal Lactate: A New Mutation in the DARS2 Gene

Leukoencephalopathy with brainstem and spinal cord involvement and elevated brain lactate diagnosis is based on its highly characteristic pattern of abnormalities observed by magnetic resonance imaging and spectroscopy. Clinically, affected patients develop slowly progressive cerebellar ataxia, spasticity, and dorsal column dysfunction, sometimes with a mild cognitive deficit or decline. In 2007, the pathophysiology of this disorder was elucidated with the discovery of mutations in the DARS2 gene, which encodes mitochondrial aspartyl-tRNA synthetase, in affected individuals. Here, the authors present a case of leukoencephalopathy with brainstem and spinal cord involvement with normal brain lactate, in which genetic analysis revealed a new mutation in the DARS2 gene not previously described.

Urinary 6-Sulphatoxymelatonin Levels Are Depressed in Chronic Migraine and Several Comorbidities

(Headache 2010;50:413‐419)

Neurocutaneous melanosis: Follow-up and literature review

Neurocutaneous melanosis is a rare, congenital, non-inherited syndrome characterized by numerous and/or large congenital nevi with intracranial leptomeningeal melanocytosis. This report describes two patients, presenting with a giant congenital nevus involving a major portion of the posterior trunk with satellite congenital nevi scattered all over the body, who developed seizures at 4 and 6 months of age, respectively. Changes in follow-up magnetic resonance (MR) examinations over an 8-year period were seen in case 1, while parenchymal melanocytic accumulation was reported in the region of the amygdala in case 2. These cases emphasize that neurocutaneous melanosis should be suspected in patients with giant congenital nevus with or without neurological symptoms. Also, neuroaxial MR screening should be performed in all cases and, ideally, before myelination of the brain to provide the highest sensitivity for detecting melanin deposits in the leptomeninges.

Sleep terrors antecedent is common in adolescents with migraine

UNLABELLEDnMigraines and sleep terrors (STs) are highly prevalent disorders with striking similarities. The aim of this study was to evaluate the effect of the antecedent of STs by comparing adolescents suffering from migraines with healthy controls in a large consecutive series.nnnMETHODSnAll patients were subjected to a detailed headache questionnaire and were instructed to keep a headache diary during a two-month period. The age range was 10 to 19 years. The diagnosis of STs was defined according to the International Classification of Sleep Disorders.nnnRESULTSnA total of 158 participants were evaluated. Of these participants, 50 suffered from episodic migraines (EMs), 57 had chronic migraines (CMs) and 51 were control subjects (CG). Participants who had a history of STs had significantly more migraines than participants who did not.nnnCONCLUSIONSnMigraine is strongly associated with a history of STs in the adolescent population independent of demographics and pain intensity.

Psychiatric symptoms may contribute to poor quality of life in adolescents with migraine

The impact of migraine on quality of life (QOL) can be aggravated by other comorbid factors. The aim of the present study was to assess the differences in the QOL of adolescents with chronic migraine, episodic migraine, and healthy adolescents, and whether the differences in QOL among the diagnostic groups were associated with the presence of self‐reported psychiatric symptoms, such as depression and anxiety.

Spontaneous periodic hypothermia and hyperhidrosis: a possibly novel cerebral neurotransmitter disorder

Spontaneous periodic episodes of hypothermia still defy medical knowledge. In 1969, Shapiro et al. described the first two cases of spontaneous periodic hypothermia associated with agenesis of the corpus callosum. Recently, Dundar et al. reported a case of spontaneous periodic hypothermia and hyperhidrosis without corpus callosum agenesis, suggesting that the periodic episodes of hypothermia might be of epileptiform origin. Here we describe two paediatric patients with spontaneous periodic hypothermia without corpus callosum agenesis and demonstrate, to our knowledge for the first time, altered levels of neurotransmitter metabolites within the cerebrospinal fluid.

Menkes disease as a differential diagnosis of child abuse

Professor and Chairman, Division of Child Neurology / Department of Neurology and Neurosurgery, UNIFESP-EPM.Received 20 October 2008, received in final form 13 January 2009. Accepted 3 April 2009.Dra Juliana Harumi Arita – Disciplina de Neuropediatria / Departamento de Neurologia e Neurocirurgia - Rua Botucatu 720 - 04023-900 Sao Paulo SP - Brasil. E-mail: julyarita@uol.com.br

Pyridoxine-dependent epilepsy initially responsive to phenobarbital

Pyridoxine-dependent epilepsy is a rare autosomal recessive disorder characterized by recurrent seizures that are not controlled by anticonvulsant medications but remits after administration of pyridoxine. We report on a 30 day-old girl who presented with seizures during the first day of life, initially responsive to anticonvulsant therapy, which remitted within two weeks. Seizures were characterized as multifocal myoclonic jerks of upper and lower limbs associated with buccal-lingual oral movements and eyelid blinking. Laboratory and neuroimaging studies were normal. Electroencephalographic record demonstrated a abnormal background activity with high-voltage epileptic discharges and a burst-suppression pattern. The seizures ceased after oral administration of pyridoxine, but recurred after withdrawal, confirming the diagnosis.

Adolescents with chronic migraine commonly exhibit depressive symptoms

Psychiatric comorbidity in patients with headache contributes to poorer prognosis, chronification of disease, poor response to treatment, increased cost of treatment, and decreased quality of life. The purpose of the present study was to evaluate the depressive symptoms in adolescents with chronic and episodic migraines and healthy adolescents. The study was performed between November 2010 and November 2011. All patients completed a detailed headache questionnaire comprising of demographical and clinical data and were instructed to fill out a headache diary over a 2-month period. The subjects ranged in age from 13 to 19xa0years. To evaluate depression symptoms, all of the subjects were asked to fill out the Beck Depression Inventory (BDI). A total of 137 participants were evaluated; 44 had episodic migraine (EM), 46 had chronic migraine (CM) and 47 were control subjects. Patients with a history of chronic migraine had significantly higher scores on the BDI than the other participants. Patients with chronic migraine had BDI scores that were 8.8 points higher than controls [95xa0% CI (β)xa0=xa05.0, 12.6] and 5.8 points higher than patients with EM [95xa0% CI (β)xa0=xa02.2, 9.4]. The main finding of this study was that chronic migraine is strongly associated with depression symptoms, regardless of demographic data. Comorbid depression may increase the total burden of migraine and diagnosis and treatment of depression in adolescents with migraine is likely to result in a better prognosis.