Clinical features of cerebral palsy in children with symptomatic congenital cytomegalovirus infection

BackgroundHuman cytomegalovirus is the most common cause of vertically transmitted viral infection, affecting around 1% of liveborns. Infection is symptomatic in nearly 10% of infected children who are at higher risk of development of severe neurological disorders, including cerebral palsy.AimsTo study the clinical profile of children with cerebral palsy caused by symptomatic congenital cytomegalovirus infection in a multicenter study involving six countries from the Surveillance of Cerebral Palsy in Europe (SCPE) Network.MethodsData on 35 children (13 males, 22 females; mean age at last assessment 12y 6mo, age range 14y 6mo, min 4y, max 18y 6mo) on pre/peri/neonatal history and last clinical assessment were collected. Classification of cerebral palsy and associated impairments was performed according to SCPE criteria.ResultsThe majority of children had bilateral spastic cerebral palsy, 85.7%, with a confidence interval (CI) [69.7–95.2], and 71.4% [CI 53.7–85.4] were unable to walk (GMFCS levels IV–V) while fine motor function was severely affected in 62.8% [CI 44.9–78.5] (BFMF levels IV and V). Most of the children with severe CP had severe associated impairments. 11.4% of children had severe visual and 42.8% severe hearing impairment, 77.1% [CI 59.9–89.6] suffered from epilepsy, also 77.1% had severe intellectual impairment, and speech was undeveloped in 71.4%. Female:male ratio was 1.69:1 and 80% of children were term born.ConclusionsCerebral palsy following symptomatic congenital cytomegalovirus infection seems to be in most cases a severe condition and associated impairments are overrepresented.     

Diagnosis and treatment of vertebral hemangiomas with neurologic deficit: a report of 29 cases and literature review

Background contextVertebral hemangiomas (VHs) are called benign tumors but are actually just vascular malformations. The diagnosis and treatment for aggressive VHs is still controversial, due to their rarity.PurposeTo evaluate the safety and efficiency of the present diagnostic methods and treatment choices.Study designA retrospective study of aggressive VHs with neurologic deficit.Patients sampleA total of 29 consecutive aggressive VH cases were diagnosed and treated in our department since 2001.Outcome measuresWe routinely took anteroposterior and lateral spinal roentgenograms, computed tomography, and magnetic resonance images.MethodsTrocar biopsy is indicated in suspected malignant cases. Radiotherapy was usually our first choice if the neurologic deficit was mild or developed slowly. Surgery was indicated if the neurologic deficit was severe or developed quickly or if the radiotherapy was not effective.ResultsThis series included 12 males and 17 females, and the mean age at diagnosis was 44.0 years (range, 21–72 years). Ten patients had radiculopathy, 1 had cauda equina syndrome, and 18 cases had myelopathy. Twenty-one cases had lesions in the thoracic spine, 5 in the lumbar, and 3 in the cervical region. Eleven cases had untypical image findings, including five cases with pathologic vertebral fracture. The neurologic compression came from only epidural soft tumor mass in 18 cases, whereas it came from both bony compression and soft lesion in the other 11 cases. Ten cases had radiotherapy alone, but two failed and had surgery later. Twenty-one cases had surgery. In the 12 cases having surgical decompression without vertebroplasty, the average estimated blood loss was 1900 mL, and it was 1093 mL for the eight cases having decompression with vertebroplasty. The average follow-up was 51.1 months (range, 24–133 months). There was no recurrence in those cases with radiotherapy, whereas three had local recurrence in those six cases treated by surgical decompression alone without radiotherapy.ConclusionsIn aggressive VHs, epidural soft-tissue compression was usually the main reason for neurologic deficit. In cases with rapid progressive and/or severe myelopathy, posterior decompression and stabilization could be combined with intraoperative vertebroplasty to reduce blood loss.     

La gestión de la asistencia neurológica en tiempos de la pandemia de Covid-19

IntroductionThe COVID-19 epidemic has led to the need for unprecedented decisions to be made to maintain the provision of neurological care. This article addresses operational decision-making during the epidemic.DevelopmentWe report the measures taken, including the preparation of a functional reorganisation plan, strategies for hospitalisation and emergency management, the use of telephone consultations to maintain neurological care, provision of care at a unit outside the hospital for priority patients, decisions about complementary testing and periodic in-hospital treatments, and the use of a specific telephone service to prioritise patients with epileptic seizures.ConclusionDespite the situation of confinement, neurology departments must continue to provide patient care through different means of operation. Like all elements of management, these must be evaluated.     

Blood harmane (1-methyl-9H-pyrido[3,4-b]indole) concentration in dystonia cases vs. controls

BackgroundHarmane (1-methyl-9H-pyrido[3,4-b]indole) (HA) is a potent neurotoxin that has been linked to two neurological diseases, essential tremor and Parkinson's disease. Blood harmane concentrations [HA] are elevated in patients with both diseases. An important question is whether HA is specifically linked with these diseases or alternatively, is a non-specific marker of neurological illness.ObjectivesWe assessed whether blood [HA] was elevated in patients with a third neurological disease, dystonia, comparing them to controls.MethodsBlood [HA] was quantified by high performance liquid chromatography. Subjects comprised 104 dystonia cases and 107 controls.ResultsMean log blood [HA] in dystonia cases was similar to that of controls (0.41 ± 0.51 g⁻¹⁰/ml vs. 0.38 ± 0.61 g⁻¹⁰/ml, t = 0.42, p = 0.68). In unadjusted and adjusted logistic regression analyses, log blood [HA] was not associated with the outcome (diagnosis of dystonia vs. control): odds ratio (OR)_unadjusted = 1.11, 95% confidence interval (CI) = 0.69–1.79, p = 0.68; OR_adjusted = 1.07, 95% CI = 0.58–1.97, p = 0.84.ConclusionsIn contrast to the elevated blood [HA] that has been reported in patients with essential tremor and Parkinson's disease, our data demonstrate that blood [HA] was similar in patients with dystonia and controls. These findings provide the first support for the notion that an elevated blood [HA] is not a broad feature of neurological disease, and may be a specific feature of certain tremor disorders.