Dataset Information

Ictal syncope or isolated syncope? A case report highlighting the overlap

ABSTRACT: Abstract

Background

Ictal bradyarrhythmia is a rare condition defined by temporal lobe epilepsy resulting in bradycardia or asystole and can result in syncope. This needs to be differentiated from isolated syncope in patients with seizure disorder, as treatment strategies differ.

Case summary

A 50-year-old female with well-controlled temporal epilepsy and a 20-year seizure-free period presented to her neurologist with abrupt onset of sudden drop attacks thought to be ictal events with potential underlying ictal bradyarrhythmia and was initially treated with escalation of anticonvulsant therapy. However, her workup was consistent with a diagnosis of cardiac syncope. She subsequently underwent successful insertion of a pacemaker, with no recurrence of her presenting episodes at a 13-month follow-up.

Discussion

Ictal syncope and isolated syncope may share a common terminal pathway and may have similar presenting symptoms. In patients with known seizure disorder, loss of consciousness may be attributable to epileptic events, ictal syncope, or isolated syncope—which can be difficult to differentiate. This case highlights the ambiguous nature of such episodes and the importance of simultaneous electroencephalogram/electrocardiogram monitoring, as this can have implications on treatment.

SUBMITTER: Fanous Y

PROVIDER: S-EPMC8573163 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Context: ROHHAD syndrome presents a significant resemblance to HIDEA syndrome. The latter is caused by biallelic loss-of-function variants in the P4HTM gene and encompasses hypotonia, intellectual disabilities, eye abnormalities, hypoventilation, and dysautonomia. We report the first patient identified with HIDEA syndrome from our ROHHAD cohort. Clinical case: Our patient was a 21-month-old girl who had a history of severe respiratory infections requiring intensive care, hypotonia, abnormal eye movements, and rapid weight gain. Polysomnography identified severe central hypoventilation. During her follow-up, a significant psychomotor delay and the absence of language were gradually observed. The prolactin levels were initially increased. Hypothermia was reported at 4 years. Exome sequencing identified a new homozygous truncating P4HTM variant. Discussion: Our patient met the diagnosis criteria for ROHHAD, which included rapid weight gain, central hypoventilation appearing after 1.5 years of age, hyperprolactinemia suggesting hypothalamic dysfunction, and autonomic dysfunction manifesting as strabismus and hypothermia. However, she also presented with severe neurodevelopmental delay, which is not a classic feature of ROHHAD syndrome. HIDEA syndrome presents similarities with ROHHAD, including hypoventilation, obesity, and dysautonomia. To date, only 14% of endocrinological disturbances have been reported in HIDEA patients. Better delineation of both syndromes is required to investigate the eventual involvement of P4HTM, a regulator of calcium dynamics and gliotransmission, in ROHHAD patients. Conclusion: In the case of clinical evidence of ROHHAD in a child with abnormal neurological development or eye abnormalities, we suggest that the P4HTM gene be systematically interrogated in addition to the analysis of the PHOX2B gene. A better delineation of the natural history of HIDEA is required to allow further comparisons between features of HIDEA and ROHHAD. The clinical similarities could potentially orient some molecular hypotheses in the field of ROHHAD research.