West Syndrome in Children With Congenital Zika Virus Infection.

Congenital Zika infection has been linked with a characteristic phenotype including neurologic sequelae. However, West syndrome has not been previously well described as a consequence. We aim to show this association through a retrospective descriptive study performed in Ecuador. Among 147 infants with congenital Zika infection, 7.5% suffered from West syndrome. Vigabatrin seems to be effective to control the spasms.

Neurological Development, Epilepsy, and the Pharmacotherapy Approach in Children with Congenital Zika Syndrome: Results from a Two-Year Follow-up Study.

Clinical outcomes related to congenital Zika syndrome (CZS) include microcephaly accompanied by specific brain injuries. Among several CZS outcomes that have been described, epilepsy and motor impairments are present in most cases. Pharmacological treatment for seizures resulting from epilepsy is performed with anticonvulsant drugs, which in the long term are related to impairments in the child's neuropsychomotor development. Here, we describe the results from a two-year follow-up of a cohort of children diagnosed with CZS related to the growth of the head circumference and some neurological and motor outcomes, including the pharmacological approach, and its results in the treatment of epileptic seizures. This paper is part of a prospective cohort study carried out in the state of Mato Grosso Sul, Brazil, based on a Zika virus (ZIKV)-exposed child population. Our data were focused on the assessment of head circumference growth and some neurological and motor findings, including the description of seizure conditions and pharmacological management in two periods. Among the 11 children evaluated, 8 had severe microcephaly associated with motor impairment and/or epilepsy. Seven children were diagnosed with epilepsy. Of these, 3 had West syndrome. In four children with other forms of epilepsy, there was no pharmacological control.

Congenital Zika Syndrome and Infantile Spasms: Case Series Study.

Recently described, the congenital Zika syndrome caused by the Zika virus has many features of other congenital infections. This case series study reports 22 infants with congenital Zika syndrome in Brazil who developed infantile spasms during their first year of life. The median age of infantile spasms onset was 4.3 (±1.4) months and ranged from 1 to 7 months. Neurologic evaluation showed that all 22 children presented severe impairment of neuropsychomotor development. A total of 18 infants had an interictal hypsarrhythmia electroencephalogram (EEG) pattern. Vigabatrin was prescribed as first-line treatment; however, only 5 patients were controlled. Steroid regimes were started for children who did not respond to vigabatrin and they are being followed. In conclusion, congenital Zika syndrome may be considered as an etiological agent of infantile spasms.

[Three cases of human herpesvirus-6 encephalopathy showing hyperperfusion in the acute phase on SPECT].

Brain hypoperfusion observed on single-photon emission computed tomography (SPECT) is a typical finding in the acute phase of human herpesvirus-6 (HHV-6) encephalopathy. However, from 2004 to 2010, we encountered three cases of HHV-6 encephalopathy in which hyperperfusion in the area of the brain lesion was observed on SPECT performed within 48 hours after disease onset. The hyperperfusion in the brain was followed by hypoperfusion in the recovery phase. These cases suggest that hyperperfusion may appear in damaged areas prior to the hypoperfusion that is normally associated with HHV-6 encephalopathy.

Infantile spasms and cytomegalovirus infection: antiviral and antiepileptic treatment.

From 1 January 1995 to 31 December 2004, 22 patients (13 males, nine females; age range 2-12mo) with infantile spasms and cytomegalovirus (CMV) infection were treated with intravenous ganciclovir (GCV) and antiepileptic drugs. GCV was given for 3 to 12 weeks with a 1-month interval (one, two, or three courses). Epileptic spasms occurred before (group A: eight patients), simultaneously (group B: eight patients), and after (group C: six patients) a diagnosis of human CMV (HCMV) infection and antiviral treatment. In 11 patients, DNA CMV [corrected] was found in cerebrospinal fluid by nested-polymerase chain reaction method (neuroinfection). All infants excreted CMV in urine. DNA CMV [corrected] and specific immunoglobulin M and immunoglobulin G antibodies were present in blood. Ten patients, including four with neuroinfection, have been seizure-free for at least the past 18 months. In two patients with neuroinfection, vigabatrin monotherapy was withdrawn after a 2 year 6 month seizure-free period. Eighteen patients required antiepileptic drugs polytherapy, four of whom required additional adrenocorticotropic hormone (ACTH). Six patients on polytherapy were seizure-free on follow-up, two of whom were treated with ACTH, but one patient [corrected] who required ACTH [corrected] was seizure-free on follow-up. In five patients, psychomotor development was normal, 16 had tetraplegia (Gross Motor Function Classification System [GMFCS] Level V), and one had diplegia (GMFCS Level III). Early antiviral and antiepileptic therapy could result in the long-term cessation of seizures.

Remission associated with human herpesvirus infection in west syndrome.

Generally, West syndrome is an intractable epileptic syndrome in infancy, although spontaneous remission has been reported in some cases. An immunologic response to infection might be one of the factors involved in the remission of West syndrome, but the mechanisms remain unknown. On the other hand, exanthema subitum is a common disease occurring in infancy with the characteristics of fever and rash. Two kinds of human herpesvirus, 6 and 7, have been isolated as causal agents of exanthema subitum. We experienced one symptomatic case and three cryptogenic cases of West syndrome that showed spontaneous remission. In the symptomatic case, the subject showed a temporary remission; however, in the other cases, the remissions were long term. In the present study, we report the patients' improvement and electroencephalographic (EEG) findings. In all of our cases, hypsarrythmia disappeared on the EEG findings, the human herpesvirus 6 IgG antibodies increased in all four cases, and the herpesvirus 7 IgG antibodies increased in two cases. We postulate that the remission of the four cases proceeded from infection by exanthema subitum. The changes in serum antibody values suggest that the spontaneous remission of West syndrome was related to human herpesvirus 6 and 7 infections.

[Clinical, neuro-radiological and prognostic aspects of post-encephalitic catastrophic epilepsies]. / Aspectos clínicos, neurorradiológicos y evolutivos de las epilepsias catastróficas postencefalíticas.

OBJECTIVE: To determine the prevalence of encephalitis and meningo encephalitis as the causative agents of catastrophic epilepsies (CE) and the incidence of post encephalitic CE, when catastrophic epilepsy is defined as often refractory to treatment and always associated with psychoneurological deterioration. PATIENTS AND METHODS: The prevalence of central nervous system (CNS) infections in determining West s syndrome (WS), Lennox Gastaut syndrome (LGS) and HHE syndrome (HHES) was detected in the large series published since 1980 in which the cause was stated. The incidence of CE in the course of meningoencephalitis was deduced from three studies done in the Virgen del Roc o Hospital: study 1 of 1,221 children admitted to hospital with the diagnosis of meningo encephalitis; study 2 of 55 cases of tuberculous meningitis; study 3 of 30 cases of encephalitis. RESULTS: CNS infections causing CE are responsible for from 3 to 11% of all WS, 3 to 8.2% of all LGS and 19% of the HHES with a catastrophic course. The commonest causes are infection due to cytomegalovirus and toxoplasmosis during the prenatal stage and the purulent meningitis, tuberculous meningitis and herpetic encephalitis during the neonatal and postnatal periods. The evidence of CE in meningo encephalitis varies according to the germ, age and severity of the aggression. CNS infections during the neonatal period in 3% of cases cause CE. In babies, newborn and subsequently, tuberculous meningitis (12.7%), measles meningo encephalitis (22%) and herpetic encephalitis (50%) lead to refractory epileptic seizures and very severe psychoneurological deterioration. CONCLUSIONS: 1. Encephalitis and meningo encephalitis are commoner than usually thought as a cause of CE. 2. They cause 3 11% of the WS, 3 8% of the LGS and 19% of the HHES. 3. The incidence of CE in the course of meningo encephalitis varies according to the germ involved and the severity of the aggression. 4. CE are very frequent during the course of herpetic encephalitis, measles meningo encephalitis and tuberculous meningo encephalitis. The latter two are becoming much less common. 5. The prognosis is extremely serious

Spontaneous remission of spasms in West syndrome--implications of viral infection.

On a database of the Japanese medical literature between 1970 and 2000, there were 29 patients with West syndrome showing spontaneous remission. In 25/29 (86%) of the patients, spontaneous remission was preceded by viral infections, among which exanthema subitum predominated. In 16/29 (55%), the spasms recurred a median of 2 months later. However, some of the remaining infants attained lasting remission of the spasms. This link between viral infections and spontaneous remission suggests immuno-inflammatory processes underlie West syndrome in certain patients. Better understanding of such mechanisms may lead to a new therapeutic approach to West syndrome.