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

• Published in: Neurology Vol. 103; no. 5; p. e209759
• Main Authors: Proietti, Jacopo, Di Censo, Rita, Spolverato, Silvia, Cossu, Alberto, Bucci, Alessandra, Plebani, Mauro, Lanterna, Andrea, Piardi, Franziska, Lo Barco, Tommaso, Cantalupo, Gaetano, Fiorini, Elena, Fontana, Elena, Dalla Bernardina, Bernardo, Francesca, Darra
• Format: Journal Article
• Language: English
• Published: United States 10-09-2024
• Subjects: Child; Electroencephalography; Female; Headache - etiology; Headache - physiopathology; Hemiplegia - diagnosis; Hemiplegia - etiology; Hemiplegia - physiopathology; Humans; Hyperventilation - complications; Hyperventilation - physiopathology
• ISSN: 0028-3878; 1526-632X; 1526-632X
• DOI: 10.1212/WNL.0000000000209759

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.