Congenital Zika Virus Infection: a Review with Emphasis on the Spectrum of Brain Abnormalities.

PURPOSE OF REVIEW: In 2016, the World Health Organization declared the Zika virus (ZIKV) outbreak a Public Health Emergency of International Concern following a cluster of associated neurological disorders and neonatal malformations. Our aim is to review the clinical and neuroimaging findings seen in congenital Zika syndrome. RECENT FINDINGS: ZIKV injures neural progenitor cells in the hippocampus, a brain region important for learning, memory, cognition, and emotion/stress response. Positron emission tomography has revealed global neuroinflammation in ZIKV infection in animal models. Congenital Zika syndrome is associated with a spectrum of brain abnormalities, including microcephaly, parenchymal calcifications, malformations of cortical development and defective neuronal migration, corpus callosum abnormalities, ventriculomegaly, and brainstem and cerebellar abnormalities.

Virtual rehabilitation via Nintendo Wii® and conventional physical therapy effectively treat post-stroke hemiparetic patients.

BACKGROUND: The Nintendo® Wii is a simple and affordable virtual therapy alternative. It may be used at home, and it is a motivating recreational activity that provides continuous feedback. However, studies comparing the use of the Nintendo® Wii to conventional physical therapy are needed. OBJECTIVE: To compare the effect of a rehabilitation treatment using the Nintendo® Wii (NW) with conventional physical therapy (CPT) to improve the sensorimotor function and quality of life for post-stroke hemiparetic patients. METHODS: The present study applied a randomized, blind, and controlled clinical trial. In total, 30 patients with post-stroke hemiparesis were evaluated. A total of 15 patients were randomly assigned to each group. The SF-36 quality of life and Fugl-Meyer scales were used to evaluate the patients. RESULTS: After treatment, the only variable that differed between the groups was the physical functioning domain of the SF-36 in the group that received conventional physical therapy. A significant difference was observed between both groups before and after treatment in terms of the following Fugl-Meyer scale items: passive movement and pain, motor function of the upper limbs (ULs), and balance. The CPT group also showed a significant difference with regard to their UL and lower limb (LL) coordination. The SF-36 scale analysis revealed a significant difference within both groups with regard to the following domains: physical functioning, role limitation due to physical aspects, vitality, and role limitation due to emotional aspects. The NW group also exhibited a significant difference in the mental health domain. The results indicate that both approaches improved the patients' performance in a similar manner. CONCLUSION: Virtual rehabilitation using the Nintendo Wii® and CPT both effectively treat post-stroke hemiparetic patients by improving passive movement and pain scores, motor function of the upper limb, balance, physical functioning, vitality, and the physical and emotional aspects of role functioning.

The Use of Nintendo Wii in the Rehabilitation of Poststroke Patients: A Systematic Review.

BACKGROUND: To evaluate the effectiveness of the video game console Nintendo Wii (NW) in motor function, balance, and functional independence in the treatment of poststroke patients and to identify which games are commonly used in therapy. METHODS: Randomized controlled trials were researched in MEDLINE, Cochrane Library, PEDro, CAPES Periodic, BIREME, and LILACS databases, covering publications up to March 31, 2014. The assessment of methodological quality was performed using the PEDro Scale as reference. RESULTS: The 5 studies included for analysis showed that NW can provide an improvement of motor function of the individual, but the data are unclear when it comes to the balance and functional independence. CONCLUSIONS: It was concluded that there is little evidence to ensure the effectiveness and support the inclusion of the treatment with NW in patients with sequelae caused by a stroke; however, some of the studies analyzed suggest that NW can provide improvement in motor function.

Migraine is frequent in children and adolescents with neurofibromatosis type 1.

BACKGROUND: Despite the high prevalence of headache in patients with neurofibromatosis type 1 (NF1), little data exist regarding the classification and characterization of headaches experienced by these patients. This paper describes a study of headache in patients with NF1 compared with healthy controls. METHODS: In this transversal study, participants (aged 4-19 years) were classified into two groups: NF1 patients or control subjects. The diagnosis of NF1 was performed according to the diagnostic criteria of the National Institutes of Health Consensus Conference, and the headache diagnosis was performed according to the diagnostic criteria of the International Classification of Headache Disorders, Second Edition. All participants underwent physical and neurologic evaluation and completed a detailed headache questionnaire. RESULTS: The comparison of 50 patients with NF1 and 50 age-matched controls revealed that the complaint of headache was significantly more frequent in the NF1 group than in the control group (CG) (62% vs 14%, χ(2)(1) = 22.4; P < 0.001). Migraine was significantly more frequent in patients with NF1 than in the CG (54% vs 14%, χ(2)(1) = 17.82; P < 0.001). No differences were found between the two groups regarding the use of simple analgesics (NF1: 14% vs CG: 5%, χ(2)(1) = 1.18; P = 0.276). CONCLUSIONS: Children and adolescents with NF1 are prone to migraines. Complaints of headache are very frequent in this population.

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

BACKGROUND: 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. METHODS: A total of 157 adolescents (aged 15-19 years old) were included in the study. Fifty patients had episodic migraine, 56 patients suffered from chronic migraine, and 51 healthy adolescents were controls. All of the participants responded to a detailed headache questionnaire, the Medical Outcomes Trust 36-Item Short-form Health Survey, the State-Trait Anxiety Inventory and the Beck Depression Inventory. RESULTS: Chronic migraine patients showed a significantly lower QOL than the control subjects in five dimensions of the Medical Outcomes Trust 36-Item Short-form Health Survey, and lower QOL than the episodic migraine patients in four dimensions. High levels of self-reported depressive symptoms were associated with lower QOL in five dimensions and high levels of self-reported anxiety were associated with lower QOL in four dimensions. CONCLUSIONS: The QOL of adolescent migraine sufferers may be aggravated not only by migraine but also by other factors, such as anxiety and depressive symptoms, which may contribute to the poor QOL in adolescents suffering from migraine.

Analysis of risk factors associated with cerebral angiography headache.

BACKGROUND: Despite previous studies indicating a moderate/high incidence of angiography headache (AH), there is still limited data about the risk factors associated with its occurrence. OBJECTIVE: The present study aimed to assess the associations among demographic, clinical, and technical characteristics of cerebral digital subtraction angiography (DSA) and the occurrence of AH. METHODS: Cross-sectional analytical observational study with a sample comprised of individuals with a recommendation for elective DSA. Clinical interviews were conducted to assess the occurrence of AH, using a standardized questionnaire. RESULTS: Among 114 subjects, the mean age was 52.8 (±13.8) years old, 75.4% (86/114) were women, 29.8% (34/114) had a history of migraines, and 10.5% (12/114) had chronic headaches. The overall frequency of AH was 45.6% (52/114). Of those, 88.4% (46/52) underwent 3D angiography, 7.7% (4/52) underwent aortography, and 1.9% (1/52) underwent both procedures. There was a statistically significant association between AH and previous history of migraine (odds ratio [OR]: 4.9; 95% confidence interval [CI] 1.62-14.7; p = 0.005) and 3D angiography (OR 6.62; 95%CI: 2.04-21.5; p = 0.002). CONCLUSIONS: 3D angiography is strongly associated with the occurrence of AH, which has never been reported before. The association between a previous history of migraine and AH confirms the results of previous studies.

ANTECEDENTES: Apesar de estudos prévios indicarem uma incidência moderada/alta de cefaleia da angiografia (CA), os dados sobre os fatores de risco associados à sua ocorrência ainda são relativamente escassos. OBJETIVO: O presente estudo teve como objetivo avaliar as associações entre as características demográficas, clínicas e técnicas da angiografia cerebral por subtração digital (ACSD) e a ocorrência de CA. MéTODOS: Estudo observacional analítico transversal com uma amostra composta por indivíduos com indicação de ACSD em caráter eletivo. Entrevistas clínicas foram realizadas utilizando um questionário padronizado para acessar a ocorrência de CA. RESULTADOS: Entre os 114 indivíduos, a idade média foi de 52,8 (±13,8) anos, 75,4% (86/114) eram mulheres, 29,8% (34/114) tinham histórico de enxaqueca e 10,5% (12/114) tinham cefaleia crônica. A frequência geral de CA foi de 45,6% (52/114). Desses, 88,4% (46/52) foram submetidos à angiografia 3D, 7,7% (4/52), à aortografia e 1,9% (1/52), aos dois procedimentos. Houve associação estatisticamente significativa entre CA e histórico prévio de enxaqueca (odds ratio [OR] 4,9; intervalo de confiança [IC] 95%: 1,62­14,7; p = 0,005) e angiografia 3D (OR 6,62; IC95%: 2,04­21,5; p = 0,002). CONCLUSõES: A angiografia 3D está fortemente associada à ocorrência de CA, o que é inédito na literatura. A associação entre um histórico de enxaqueca e a CA confirma os resultados de estudos anteriores.

Pediatric central nervous system tumors: a single-center experience from 1989 to 2009.

The objective of this study was to determine the epidemiology of primary tumors of the central nervous system (CNS) in pediatric patients from a Brazilian oncology institute. We retrospectively analyzed 741 charts (415 males and 326 females) of patients under 21 years of age who were diagnosed with a CNS tumor. The analysis included patients from 1989 to 2009 and was performed using the World Health Organization criteria. We evaluated the distribution of age, sex, topography, clinical symptoms, symptom intervals, and classification of the tumors. Patients with clinical/radiologic diagnoses were included. Seven hundred forty-one patients with tumors in the CNS were reviewed, and 83% of the patients presented a histologic diagnosis. Males (56%) were more prevalent than females. In children under the age of 1 year, the supratentorial compartment was the predominant region involved (62.0%). Astrocytoma was the most frequent tumor type (37.0%), followed by medulloblastoma (13.6%), craniopharyngioma (10.5%), and ependymoma (6.8%). Headaches were the most common symptom, and the symptom intervals varied from 1 to 5010 days. Approximately 4% of the patients had associated genetic syndromes. Although it was not a population study and selection bias may have occurred, this study supplies important epidemiologic data from an emerging country in which population studies are rare.

Electrical status epilepticus during sleep in patients with congenital Zika virus syndrome: An unprecedented clinical finding.

BACKGROUND: Brazil experienced a disproportionately higher rate of microcephaly cases in November 2015 with evidence of a causal link with Zika virus (ZIKV) infections during pregnancy. Epilepsy is a major neurological feature seen as part of congenital Zika virus syndrome (CZVS). Different seizure types and electroencephalographic (EEG) abnormalities have been described in association with this syndrome. However, clinical and neurophysiological features of epilepsy seen in children with CZVS are not fully understood. METHODS: We evaluated children with CZVS showing an EEG pattern of electrical status epilepticus during slow-wave sleep (ESES). Information on gender, age of onset of seizures, head circumference at birth, gross motor function at the time of diagnosis, of clinical and EEG aspects of seizures, EEG features and response to drug treatment was assessed. RESULTS: Our case series included four patients. They were diagnosed with epilepsy between one month to 18 months of age and showed an ESES pattern at the age of three. They presented with a wide range of epileptic symptoms, but all experienced tonic seizures. Multiple drug treatment was the management approach for three patients; however, they showed poor response to treatment with conventional drugs used in the treatment of ESES. CONCLUSIONS: Children with CZVS may develop an EEG pattern of ESES. Clinicians and neurologists should be aware of this neurological presentation to improve the management of these patients.

Benign neonatal sleep myoclonus.

Benign neonatal sleep myoclonus is a non-epileptic disorder. This phenomenon of the first weeks of life is characterized by erratic myoclonic jerks occurring only during sleep and with no electroencephalographic changes. It is not associated with perinatal complications, disappears spontaneously within two to four months, and it does not compromise future development. We illustrate with a video this relatively frequent condition, which is often misdiagnosed as epileptic in nature, and discuss the clinical characteristics and differential diagnosis.

Trigger factors in migraine patients.

BACKGROUND: Migraine is a chronic neurological disease with several trigger factors, including dietary, hormonal and environmental factors. PURPOSE: To analyse precipitating factors in a sample of migraine patients. METHOD: Two hundred consecutive migraine patients were interviewed about possible trigger factors for migraine attacks. RESULTS: Most patients showed at least one dietary trigger, fasting was the most frequent one, followed by alcohol and chocolate. Hormonal factors appeared in 53%, being the pre-menstrual period the most frequent trigger. Physical activities caused migraine in 13%, sexual activities in 2.5% and 64% reported emotional stress a trigger factor. 81% related some sleep problem as a trigger factor. Regarding environmental factors, smells were reported by 36.5%. CONCLUSION: Trigger factors are frequent in migraine patients, its avoidance may decrease headache frequency and also improve patients' quality of life.

Analysis of risk factors associated with cerebral angiography headache / Análise de fatores de risco associados a cefaleia da angiografia cerebral

Abstract Background Despite previous studies indicating a moderate/high incidence of angiography headache (AH), there is still limited data about the risk factors associated with its occurrence. Objective The present study aimed to assess the associations among demographic, clinical, and technical characteristics of cerebral digital subtraction angiography (DSA) and the occurrence of AH. Methods Cross-sectional analytical observational study with a sample comprised of individuals with a recommendation for elective DSA. Clinical interviews were conducted to assess the occurrence of AH, using a standardized questionnaire. Results Among 114 subjects, the mean age was 52.8 (±13.8) years old, 75.4% (86/114) were women, 29.8% (34/114) had a history of migraines, and 10.5% (12/114) had chronic headaches. The overall frequency of AH was 45.6% (52/114). Of those, 88.4% (46/52) underwent 3D angiography, 7.7% (4/52) underwent aortography, and 1.9% (1/52) underwent both procedures. There was a statistically significant association between AH and previous history of migraine (odds ratio [OR]: 4.9; 95% confidence interval [CI] 1.62-14.7; p = 0.005) and 3D angiography (OR 6.62; 95%CI: 2.04-21.5; p = 0.002). Conclusions 3D angiography is strongly associated with the occurrence of AH, which has never been reported before. The association between a previous history of migraine and AH confirms the results of previous studies.

Resumo Antecedentes Apesar de estudos prévios indicarem uma incidência moderada/alta de cefaleia da angiografia (CA), os dados sobre os fatores de risco associados à sua ocorrência ainda são relativamente escassos. Objetivo O presente estudo teve como objetivo avaliar as associações entre as características demográficas, clínicas e técnicas da angiografia cerebral por subtração digital (ACSD) e a ocorrência de CA. Métodos Estudo observacional analítico transversal com uma amostra composta por indivíduos com indicação de ACSD em caráter eletivo. Entrevistas clínicas foram realizadas utilizando um questionário padronizado para acessar a ocorrência de CA. Resultados Entre os 114 indivíduos, a idade média foi de 52,8 (±13,8) anos, 75,4% (86/114) eram mulheres, 29,8% (34/114) tinham histórico de enxaqueca e 10,5% (12/114) tinham cefaleia crônica. A frequência geral de CA foi de 45,6% (52/114). Desses, 88,4% (46/52) foram submetidos à angiografia 3D, 7,7% (4/52), à aortografia e 1,9% (1/52), aos dois procedimentos. Houve associação estatisticamente significativa entre CA e histórico prévio de enxaqueca (odds ratio [OR] 4,9; intervalo de confiança [IC] 95%: 1,62-14,7; p = 0,005) e angiografia 3D (OR 6,62; IC95%: 2,04-21,5; p = 0,002). Conclusões A angiografia 3D está fortemente associada à ocorrência de CA, o que é inédito na literatura. A associação entre um histórico de enxaqueca e a CA confirma os resultados de estudos anteriores.

Oromandibular Dystonia is a Prominent Feature in Patients with Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency

Abstract Aromatic L-Amino acid decarboxylase (AADC) deficiency is a rare neurometabolic disorder due to a homozygous or compound heterozygous pathogenic variant of the DDC gene, resulting in low synthesis of the biogenic amines dopamine, serotonin, epinephrine, and norepinephrine. Most patients had severe expression of the disease with global developmental delay, early hypotonia, movement disorders such as oculogyric crises, tremor, and dystonia. Oromandibular dystonia (OMD) is rarely recognized in patients with AADC deficiency. The aim of this study was to describe OMD in detail in 4 patients with AADC deficiency. OMD occurred in isolated form or in association with oculogyric crises, increasing the difficulty in care patients during the crises. The main form of OMD was tongue dystonia associated with mouth opening dystonia. AADC deficiency must be included in the list of genetic causes of OMD.

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.

Detection of Cerebral Vasculopathy by Transcranial Doppler in Children With Neurofibromatosis Type 1.

Neurofibromatosis type 1 is characterized by nerve sheath neurofibromas associated with a number of additional clinical features, including cerebrovascular disease. The aim of this study was to use transcranial Doppler as a screening method for identifying cerebral vasculopathy in children with neurofibromatosis type 1. Forty children with neurofibromatosis type 1, aged 5 to 18 years old, were examined by transcranial Doppler. Patients presenting with hemodynamic features of arterial stenosis/occlusion on transcranial Doppler underwent magnetic resonance angiography to confirm the findings. Magnetic resonance angiography was performed on 4 children who exhibited a transcranial Doppler hemodynamic pattern indicative of cerebral vasculopathy. Among these cases, 2 presented internal carotid artery stenosis/occlusion, 1 had bilateral middle cerebral artery stenosis, and 1 presented a normal magnetic resonance angiography result. Transcranial Doppler can be used routinely in the investigation of cerebrovascular disease in neurofibromatosis type 1 patients, where magnetic resonance angiography can be subsequently applied to confirm the diagnosis, further contributing to the prevention of cerebrovascular events.

Fibrodysplasia ossificans progressiva: case report.

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder characterized by postnatal progressive heterotopic ossification of the connective tissue and congenital malformation of the big toes. We report on a nine-year-old girl with clinical and radiological features of FOP. She was born with bilateral hallux valgus and at the age of nine presented an indurate mass in the left cervical region that was painful. A significant decreased range of motion in all levels of the spine and shoulder girdle was found. The radiographs showed heterotopic ossification in the thoracic region. The patient had two outbreaks of the disease ("flare-ups") that were treated with prednisone 2 mg/kg/day for four days. After the "flare-ups", she had a continuous therapy with a Cox-2 inhibitor (25 mg/day) and a leukotriene inhibitor, montelukast (10 mg/day).

[Chorea: a rare manifestation of Takayasu's arteritis]. / Coreia: uma manifestação rara da arterite de Takayasu.

The case of a girl with recurring chorea and a Takayasu's arteritis diagnosis is reported. This clinical manifestation has been reported in only one patient with this vasculitis in the pediatric group.

Inherited manganism: the "cock-walk" gait and typical neuroimaging features.

Manganese (Mn) toxicity causes an extrapyramidal, parkinsonian-type movement disorder with characteristic magnetic resonance images of Mn accumulation in the basal ganglia. This letter highlights the neurological manifestations and neuroimaging features of inherited manganism (IMn), an unusual and treatable inborn error of Mn homeostasis. Early-onset dystonia with "cock-walk" gait and hyperintense signal in basal ganglia, associated to polycythemia, chronic liver disease and hypermanganesemia, promptly suggest IMn, and a genetic evaluation should be performed.