Early epilepsy in children with Zika-related microcephaly in a cohort in Recife, Brazil: Characteristics, electroencephalographic findings, and treatment response.

OBJECTIVE: To estimate the incidence of epilepsy in children with Zika-related microcephaly in the first 24 months of life; to characterize the associated clinical and electrographic findings; and to summarize the treatment responses. METHODS: We followed a cohort of children, born during the 2015-2016 Zika virus (ZIKV) epidemic in Brazil, with congenital microcephaly and evidence of congenital ZIKV infection on neuroimaging and/or laboratory testing. Neurological assessments were performed at ≤3, 6, 12, 15, 18, 21, and 24 months of life. Serial electroencephalograms were performed over the first 24 months. RESULTS: We evaluated 91 children, of whom 48 were female. In this study sample, the cumulative incidence of epilepsy was 71.4% in the first 24 months, and the main type of seizure was infantile spasms (83.1%). The highest incidence of seizures occurred between 3 and 9 months of age, and the risk remained high until 15 months of age. The incidence of infantile spasms peaked between 4 and 7 months and was followed by an increased incidence of focal epilepsy cases after 12 months of age. Neuroimaging results were available for all children, and 100% were abnormal. Cortical abnormalities were identified in 78.4% of the 74 children evaluated by computed tomography and 100% of the 53 children evaluated by magnetic resonance imaging. Overall, only 46.1% of the 65 children with epilepsy responded to treatment. The most commonly used medication was sodium valproate with or without benzodiazepines, levetiracetam, phenobarbital, and vigabatrin. SIGNIFICANCE: Zika-related microcephaly was associated with high risk of early epilepsy. Seizures typically began after the third month of life, usually as infantile spasms, with atypical electroencephalographic abnormalities. The seizure control rate was low. The onset of seizures in the second year was less frequent and, when it occurred, presented as focal epilepsy.

Longitudinal evolution of electroencephalogram (EEG): Findings over five years of follow-up in children with Zika-related microcephaly from the Microcephaly Epidemic Research Group Pediatric Cohort (2015-2020).

OBJECTIVE: To assess the longitudinal evolution of EEG findings in children with Zika related-microcephaly (ZRM) and to evaluate the associations of these patterns with the children's clinical and neuroimaging characteristics. METHODS: As part of the follow-up of the Microcephaly Epidemic Research Group Pediatric Cohort (MERG-PC) in Recife, Brazil, we performed serial EEG recordings in a subgroup of children with ZRM to evaluate changes in background rhythms and epileptiform activity (EA). Latent class analysis was used to identify patterns in the evolution of EA over time; clinical and neuroimaging findings were compared across the identified groups. RESULTS: Out of the 72 children with ZRM who were evaluated during 190 EEGs/videoEEGs, all participants presented with abnormal background activity, 37.5% presented with an alpha-theta rhythmic activity, and 25% presented with sleep spindles, which were less commonly observed in children with epilepsy. EA changed over time in 79.2% of children, and three distinct trajectories were identified: (i) multifocal EA over time, (ii) no discharges/focal EA evolving to focal/multifocal EA, and (iii) focal/multifocal EA evolving to epileptic encephalopathy patterns (e.g., hypsarrhythmia or continuous EA in sleep). The multifocal EA over time trajectory was associated with periventricular and thalamus/basal ganglia calcifications, brainstem and corpus callosum atrophy and had less focal epilepsy, whereas the children in the trajectory which evolved to epileptic encephalopathy patterns had more frequently focal epilepsy. SIGNIFICANCE: These findings suggest that, in most children with ZRM, trajectories of changes in EA can be identified and associated with neuroimaging and clinical features.

Thunderclap headache attributed to reversible cerebral vasoconstriction: view and review.

Thunderclap headache attributed to reversible cerebral vasoconstriction (THARCV) is a syndrome observed in a number of reported cases. In this article we reviewed this new headache entity (idiopathic form) using the clinical-radiological findings of 25 reported patients. In this series of patients 72% were women, the mean age at the onset of first headache episode was 39.4 +/- 2.3 years. In addition to the sine qua non condition of being abrupt and severe (thunderclap) at the onset, the headache was usually described as being explosive, excruciating, or crushing. The feature of pulsatility, accompanied or not by nausea was described by 80% of the patients. Forty percent of the cases manifested vomiting and 24% photophobia. Usually the headache was generalized, and in three cases it was unilateral at least at the onset. In 21 of 25 patients (84%) there was at least one recurrence or a sudden increase in the intensity of the headache. A past history of migraine was present in 52% of the patients. Precipitating factors were identified in 56% of the patients. Sexual intercourse was described by six patients. Of the 25 patients with THARCV syndrome studied, 12 (48%) developed focal neurological signs, transitory ischemic attack (n = 1), or ischemic stroke (n = 11, 44%), and two (8%) of them manifested seizures. The THARCV syndrome is a neurological disturbance perhaps more frequent than expected, preferentially affecting middle aged female migraineurs, and having an unpredictable prognosis, either showing a benign course or leading to stroke.

Hemicrania continua secondary to an ipsilateral brainstem lesion.

We describe a 47-year-old woman with a 3-year history of a continuum mild-moderate right-side headache, with exacerbations, associated with stabbing volleys of pain on right orbit-temporal region (10/10) and right eye ptosis and lacrimation with conjunctival injection. The pain was completely abolished with indomethacin (100 mg per day). The diagnosis of hemicrania continua was made according to the International Headache Society (IHS) criteria. The headache presentation was precipitated by a stroke and a right-side brainstem lesion was present at magnetic resonance imaging. This case report shows anatomoclinical evidence of the involvement of brainstem structures on the pathophysiology of hemicrania continua.

Cluster headache and intracranial aneurysm.

In the present study we describe the cases of two patients with cluster-like headache related to intracranial carotid artery aneurysm. One of these patients responded to verapamil prescription with headache resolution. In both cases the surgical clipping of the aneurysm resolved the cluster pain. These findings strongly suggest a pathophysiological link between the two conditions. The authors discuss the potential pathophysiological mechanisms underlying cluster-like headache due to intracranial carotid artery aneurysm.

Epilepsia do lobo temporal mesial associada à esclerose hipocampal / Mesial temporal lobe epilepsy with hippocampal sclerosis

INTRODUÇÃO: A importância clínica da epilepsia do lobo temporal mesial (ELTM) decorre de sua alta prevalência e elevada proporção de pacientes com crises epilépticas refratárias ao tratamento medicamentos; sendo a esclerose mesial a etiologia encontrada em 50-70 por cento dos pacientes com ELTM refratária ao tratamento clínico. OBJETIVO: Revisão, atualização e discussão dos aspectos clínicos, de histologia e fisiopatogenia da ELTM associada à esclerose hipocampal. RESULTADOS: Apesar da relação entre esclerose hippocampal e ELTM já estar bem estabelecida na literatura, o mecanismo exato pelo qual a esclerose hipocampal participa da gênese das crises epilépticas ainda não foi completamente desvendado. CONCLUSÕES: Estudos retrospectivos de centros de cirurgia de epilepsia enfatizam a associação entre esclerose hipocampal e história de injúria precipitante inicial, tais como crises epilépticas, ocorrendo em fase precoce do desenvolvimento cerebral. Apenas recentemente fatores genéticos foram implicados na gênese da esclerose hipocampal.