Clinical Reasoning: Hyperventilation-Induced Alternating Hemiplegia With Concomitant Hemispheric EEG Slowing in a 7-Year-Old Girl With Headache.

A 7-year-old right-handed girl presented to the pediatric neurology outpatient clinic after 5 episodes of headache over the previous 3 months. Her family history was positive for migraine in the mother and maternal grandmother and for febrile seizures in the older sister. The neurologic examination and cognitive profile were normal. Five seconds after the end of hyperventilation, video-EEG showed high-amplitude delta waves predominantly over the left hemisphere with concomitant acute aphasia and right-sided weakness. After the event, which self-resolved over 8 minutes, the girl showed intact recall. A second instance of hyperventilation evoked the appearance of pseudo-rhythmic slow activity localized to the right hemisphere, associated with left-sided weakness, 20 seconds after the end of the test. This event spontaneously resolved in 3 minutes and was followed by headache.An exaggerated physiologic response to hyperventilation, the possible epileptic nature of the events, and a migraine variant were all considered in the differential. Nonetheless, the EEG slowing is shorter in duration and generalized in physiologic and paraphysiological conditions. A clear ictal morphology and evolution of the EEG activity were lacking in this case, and migraine attacks induced by hyperpnea have not been reported to date. Instead, EEG alterations similar to that observed in our patient are described in association with vascular abnormalities. We report the clinical presentation and diagnostic workup of a rare cerebrovascular disorder, highlighting the key features in the differential. Our case emphasizes the clinical value of the EEG rebuild-up phenomenon, which can help the clinician in achieving a prompt diagnosis.

Comparison of alternative gestational age assessment methods in a low resource setting: a retrospective study.

BACKGROUND: Accurate gestational age (GA) determination allows correct management of high-risk, complicated or post-date pregnancies and prevention or anticipation of prematurity related complications. Ultrasound measurement in the first trimester is the gold standard for GA determination. In low- and middle-income countries elevated costs, lack of skills and poor maternal access to health service limit the availability of prenatal ultrasonography, making it necessary to use alternative methods. This study compared three methods of GA determination: Last Normal Menstrual Period recall (LNMP), New Ballard Score (NBS) and New Ballard Score corrected for Birth Weight (NBS + BW) with the locally available standard (Ultrasound measurement in the third trimester) in a low-resource setting (Tosamaganga Council Designated Hospital, Iringa, Tanzania). METHODS: All data were retrospectively collected from hospital charts. Comparisons were performed using Bland Altman method. RESULTS: The analysis included 70 mother-newborn pairs. Median gestational age was 38 weeks (IQR 37-39) according to US. The mean difference between LNMP vs. US was 2.1 weeks (95% agreement limits - 3.5 to 7.7 weeks); NBS vs. US was 0.2 weeks (95% agreement limits - 3.7 to 4.1 weeks); NBS + BW vs. US was 1.2 weeks (95% agreement limits - 1.8 to 4.2 weeks). CONCLUSIONS: In our setting, NBS + BW was the least biased method for GA determination as compared with the locally available standard. However, wide agreement bands suggested low accuracy for all three alternative methods. New evidence in the use of second/third trimester ultrasound suggests concentrating efforts and resources in further validating and implementing the use of late pregnancy biometry for gestational age dating in low and middle-income countries.

Efficacy and safety of Fenfluramine hydrochloride for the treatment of seizures in Dravet syndrome: A real-world study.

OBJECTIVE: Dravet syndrome (DS) is a drug-resistant, infantile onset epilepsy syndrome with multiple seizure types and developmental delay. In recently published randomized controlled trials, fenfluramine (FFA) proved to be safe and effective in DS. METHODS: DS patients were treated with FFA in the Zogenix Early Access Program at four Italian pediatric epilepsy centers. FFA was administered as add-on, twice daily at an initial dose of 0.2 mg/kg/d up to 0.7 mg/kg/d. Seizures were recorded in a diary. Adverse events and cardiac safety (with Doppler echocardiography) were investigated every 3 to 6 months. RESULTS: Fifty-two patients were enrolled, with a median age of 8.6 years (interquartile range [IQR] = 4.1-13.9). Forty-five (86.5%) patients completed the efficacy analysis. The median follow-up was 9.0 months (IQR = 3.2-9.5). At last follow-up visit, there was a 77.4% median reduction in convulsive seizures. Thirty-two patients (71.1%) had a ≥50% reduction of convulsive seizures, 24 (53.3%) had a ≥75% reduction, and five (11.1%) were seizure-free. The most common adverse event was decreased appetite (n = 7, 13.4%). No echocardiographic signs of cardiac valvulopathy or pulmonary hypertension were observed. There was no correlation between type of genetic variants and response to FFA. SIGNIFICANCE: In this real-world study, FFA provided a clinically meaningful reduction in convulsive seizure frequency in the majority of patients with DS and was well tolerated.