Neuropediatrics 2023; 54(S 01): S1-S32
DOI: 10.1055/s-0043-1777185
Bewegungsstörungen

Benign Tonic Downgaze of Infancy (Pseudo-Sunset Phenomenon)

K. Haas-Lude
1   Department of Pediatric Neurology and Developmental Medicine, University Children‘s Hospital Tubingen Germany, Tübingen, Deutschland
,
C. Schramm
2   Department of Ophthalmology, University Eye Hospital Tubingen, Tübingen, Deutschland
,
M. Alber
1   Department of Pediatric Neurology and Developmental Medicine, University Children‘s Hospital Tubingen Germany, Tübingen, Deutschland
,
S. Groeschel
1   Department of Pediatric Neurology and Developmental Medicine, University Children‘s Hospital Tubingen Germany, Tübingen, Deutschland
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    Background/Purpose: Benign tonic downgaze is a very rare distinctive ocular motility disorder typically manifesting in the first months of life. It is characterized by an unusual downward deviation of the eyes. This benign (pseudo sunset) phenomenon of the eyes is usually transient and subsides within weeks or months. The pathophysiology is unknown, but a delay of maturation of the gaze reflex systems is suspected.

    Methods: Case report and video (with EEG) of a full-term girl aged 5 months. Neurological and ophthalmological examinations were conducted at initial presentation and on follow-up. Cranial ultrasound and EEG were performed at initial presentation.

    Results: The girl presented abnormal eye movements starting at the age of 2.5 months. Frequent brief episodes lasting up to 2 seconds with conjugated eye movements with bilateral downgaze occurred while awake and only in supine position. Ophthalmological examination revealed myopia of the right and emmetropia of the left eye. Optic discs showed slightly enlarged excavation. Cranial ultrasound showed no abnormalities, especially no hydrocephalus. Neurological examination and electroencephalography were normal too and there was no major developmental delay. Head circumference was below the 3rd percentile but without any dynamics in the course. At the age of 7 months, episodes with downgaze became less frequent and were not seen during examination. The ophthalmological results showed no changes over time.

    Conclusion: The clinical picture and course is typical for benign tonic downgaze of infancy. A comprehensive ophthalmic evaluation is necessary as well as a neurological assessment to exclude an underlying neurological disorder that may mimic benign tonic downgaze. It is important to recognize this benign (pseudo-sunset) phenomenon of the eyes, to avoid unnecessary investigations.


     

    No conflict of interest has been declared by the author(s).

    Publication History

    Article published online:
    13 November 2023

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