Comparative quantitative clinical, neuroimaging, and functional profiles in children with acute flaccid myelitis at acute and convalescent stages of disease.

AIM: To quantify characteristics in acute flaccid myelitis (AFM) at acute and convalescent stages. METHOD: This was a retrospective case series of children with AFM evaluated at a single institution in the USA (2014-2017). Acute inflammatory/ischemic myelopathies were excluded. Neurological assessments and segmental quantitative analysis of signal abnormalities on magnetic resonance imaging (MRI) of the brain and spinal cord were performed. RESULTS: Sixteen patients (11 males, five females) were evaluated. Median age at onset was 4 years (interquartile range [IQR] 3-6y). All had parainfectious acute-onset limb weakness, lower motor neuron examination, and spinal fluid pleocytosis. On acute spinal cord MRI, longitudinally extensive T2 hyperintensities were identified throughout the spinal cord mostly within grey matter; five out of 12 patients had dorsal brainstem T2 hyperintensities. At a median of 2 months follow-up (IQR 2-3mo), spinal cord MRI improved in seven out of nine patients although focal T2 hyperintensities persisted in cervical and lumbar grey matter. At a median follow-up of 4 months (IQR 2-6mo), Medical Research Council sum score rose from a median of 29 to 32; distal muscle groups improved more than proximal ones; four out of 16 patients were ventilator-dependent; and two out of 16 patients were quadriplegic. INTERPRETATION: While patients may show marked improvement on neuroimaging from acute to convalescent stages, the majority of children with AFM have limited motor recovery and continued disability. Clinicians should consider the timing of clinical and neuroimaging exams when assessing diagnosis and prognosis. WHAT THIS PAPER ADDS: During the 2014 to 2017 acute flaccid myelitis outbreak in the USA, clinical recovery was better in distal than proximal muscle groups. Lumbar spinal cord showed more residual abnormalities at convalescence.

Gait Disturbance as the Presenting Symptom in Young Children With Anti-NMDA Receptor Encephalitis.

This case series demonstrates a novel clinical phenotype of gait disturbance as an initial symptom in children <3 years old with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Anti-NMDAR encephalitis is one of the most common causes of encephalitis in children, more common than any of the viral encephalitides and the second most common autoimmune cause after acute disseminated encephalomyelitis. Anti-NMDAR encephalitis in children often presents with disrupted speech and sleep patterns followed by progression to motor dysfunction, dyskinesias, and seizures. Because this condition can present initially with vague symptoms, diagnosis and treatment of anti-NMDAR encephalitis are often delayed. Although nearly 40% of all reported patients are <18 years old, few infants and toddlers have been reported with this disease. Four children <3 years old were diagnosed with anti-NMDAR encephalitis at our institution. Interestingly, each child presented initially with the chief concern of gait disturbance. One child presented with unsteady walking and slurred speech, suggestive of cerebellar ataxia, and 3 had inability to bear weight on a unilateral lower extremity, resulting in unsteady gait. Two of these children had seizures at the time of hospital presentation. All developed classic behavioral changes, insomnia, dyskinesias, or decreased speech immediately before or during hospitalization. When seen in the setting of other neurologic abnormalities, gait disturbance should raise the concern for anti-NMDAR encephalitis in young children. The differential diagnosis for gait disturbance in toddlers and key features suggestive of anti-NMDAR encephalitis are reviewed.

Clinical and neuroimaging features as diagnostic guides in neonatal neurology diseases with cerebellar involvement.

Cerebellar abnormalities are encountered in a high number of neurological diseases that present in the neonatal period. These disorders can be categorized broadly as inherited (e.g. malformations, inborn errors of metabolism) or acquired (e.g. hemorrhages, infections, stroke). In some disorders such as Dandy-Walker malformation or Joubert syndrome, the main abnormalities are located within the cerebellum and brainstem. In other disorders such as Krabbe disease or sulfite oxidase deficiency, the main abnormalities are found within the supratentorial brain, but the cerebellar involvement may be helpful for diagnostic purposes. In In this article, we review neurological disorders with onset in the neonatal period and cerebellar involvement with a focus on how characterization of cerebellar involvement can facilitate accurate diagnosis and improved accuracy of neuro-functional prognosis.

Evaluation of blood pressure measurement using a miniature blood pressure transmitter with jacketed external telemetry in cynomolgus monkeys.

INTRODUCTION: Current techniques used to accurately determine arterial blood pressure (BP) in conscious, unrestrained monkeys require invasive telemetry. This study evaluated the functionality of an implanted miniature telemetry blood pressure transmitter for the collection of BP measurements in conjunction with electrocardiographic measurements using a jacketed external telemetry (JET) system in conscious, unrestrained cynomolgus monkeys. METHODS: Twenty-four animals were surgically implanted with the transmitter in the right femoral artery. Local tolerability to the implant, signal quality, and variability in hemodynamic values were evaluated. On alternate weeks, animals were given single doses of positive control agents to produce hypotensive (clonidine hydrochloride) or hypertensive (L-NAME) effects. Undisturbed telemetry BP data were continuously collected for at least 24h following dosing and analyzed. RESULTS: While exhibiting remarkably high signal quality ( approximately 95% data points retained over 24h of data collection) and moderate variability across study weeks in baseline pulse height measurements (changes as small as < 0 mmHg), nine of 18 transmitters were nonfunctional by 19 weeks post-surgery, most likely due to migration of the catheter out of the artery. In animals given positive control agents, L-NAME induced a statistically significant increase (> or = + 8 mmHg) and clonidine hydrochloride induced a statistically significant decrease (-11 mmHg) in mean arterial pressures. Histological analysis revealed femoral arterial thickening near the sites of implantation. DISCUSSION: These results demonstrate the ability of the miniature BP transmitter, in conjunction with the JET system, to detect small changes in hemodynamic data continuously collected in conscious unrestrained monkeys. Future optimization of the transmitter includes the addition of a suture rib to the transmitter body and increased catheter size to prevent catheter migration out of the artery, the root cause of failed transmitters. The miniature blood pressure transmitter evaluated provides a minimally invasive technique for continuous collection of hemodynamic data in a toxicology study environment.

Piloting the Family Check-Up With Incarcerated Adolescents and Their Parents.

The purpose of the current investigation was to pilot the Family Check-Up (FCU; see T. J. Dishion & K. Kavanagh, 2003) with 10 incarcerated adolescents and their parents or guardians. The FCU is based on principles of motivational interviewing (W. R. Miller & S. Rollnick, 2002). The authors delivered FCU with a high degree of fidelity and adherence on the basis of ratings from parents, therapists, and observers. Results suggest that the FCU positively impacted families (effect sizes were generally in the medium range). After our intervention, adolescents were more confident in their ability to resist drug use, and parents were more confident in their ability to impact their adolescents' risky behaviors. Parents and adolescents both reported being highly satisfied with this intervention. These results warrant further investigation of the FCU with incarcerated adolescents and their families in a controlled clinical trial.