An Infant Intracranial Tumor With Nystagmus

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An Infant Intracranial Tumor With Nystagmus

Did you know that sudden, involuntary eye shaking in infants could be a warning sign of a serious brain condition? A 2020 case study from Zhejiang University’s Children’s Hospital highlights why pediatricians, ophthalmologists, and parents should never overlook this symptom—and how early detection can change outcomes.

The case centers on a 4-month-old girl brought to the hospital for “sudden eyeball shaking” that lasted a week. She was a full-term, healthy infant with no history of trauma, surgery, or family risk factors. An ophthalmological exam found bilateral horizontal nystagmus (repeated, back-and-forth eye movements) but normal light-chasing responses and anterior eye structures. After dilating her pupils, a wide-angle retinal scan revealed clear optic discs and flat retinas in both eyes—but the left eye showed unusual retinal blood vessel dilation and mild distortion. A skull MRI confirmed the cause: a large tumor in the sellar region (the area around the pituitary gland) that had spread to the skull base, spinal canal, and caused supratentorial hydrocephalus (fluid buildup in the brain).

The girl underwent tumor resection at the hospital’s neurosurgery department. Post-operative pathology identified the tumor as a pilomyxoid astrocytoma (PMA), a rare central nervous system tumor that mainly affects infants and young children. PMA typically forms in the sellar region (near the optic chiasm, which connects the eyes to the brain) with a mean age of diagnosis at 18 months—making this 4-month-old’s case earlier than average. The tumor cells were loose, star-shaped, and grew around blood vessels; immunohistochemistry (tissue testing) showed markers like glial fibrillary acidic protein (GFAP) and oligodendrocyte transcription factor-2 (Olig2), consistent with PMA.

Why did the tumor cause nystagmus? Nystagmus is split into two types: infantile (appearing in the first 3–6 months, often benign) and acquired (starting later, linked to underlying issues). The girl’s nystagmus was acquired—caused by the sellar tumor blocking nerve pathways that control steady gaze. Studies show acquired nystagmus in young children is often age-dependent: younger kids with chiasmatic (optic chiasm) tumors are more likely to develop it, as reported by Lavery et al. in Ophthalmology. Ehrt et al. added that acquired nystagmus in children may also come with symptoms like oscillopsia (feeling like the world is moving), asymmetric eye movements, or neurological signs like vertigo—though this case focused on eye changes alone.

For clinicians, the case underscores a critical lesson: never stop at a routine anterior eye exam. The girl’s key clue—retinal vasodilation in the left eye—was only found with a detailed fundus (retina) scan. “Pediatricians and ophthalmologists must look for signs of acquired nystagmus and refer patients for neurological imaging,” the study authors note. For parents, it’s a reminder that sudden, unexplained eye movements in infants deserve prompt medical attention—even if other symptoms seem mild.

The study was led by Li-Li Ge and Cai-Ping Shi from the Department of Ophthalmology at The Children’s Hospital, School of Medicine, Zhejiang University, Hangzhou, China. The patient’s guardians provided written consent for the use of her medical images and information.

References

  1. Komotar RJ, Burger PC, Carson BS, et al. Pilocytic and pilomyxoid hypothalamic/chiasmatic astrocytomas. Neurosurgery. 2004;54:72–80. doi: 10.1227/01.neu.0000097266.89676.25
  2. Bresson D, Herman P, Polivka M, Froelich S. Sellar lesions/pathology. Otolaryngologic Clinics of North America. 2016;49:63–93. doi: 10.1016/j.otc.2015.09.004
  3. Abadi RV. Mechanisms underlying nystagmus. Journal of the Royal Society of Medicine. 2002;95:231–234. doi: 10.1258/jrsm.95.5.231
  4. Lavery MA, O’Neill JF, Chu FC, Martyn LJ. Acquired nystagmus in early childhood: a presenting sign of intracranial tumor. Ophthalmology. 1984;91:425–453. doi: 10.1016/s0161-6420(84)34269-5
  5. Ehrt O. Infantile and acquired nystagmus in childhood. European Journal of Paediatric Neurology. 2012;16:567–572. doi: 10.1016/j.ejpn.2012.02.010

Original study: Ge LL, Shi CP. An infant intracranial tumor with nystagmus. Chinese Medical Journal. 2020;133:99–100. doi: doi.org/10.1097/CM9.0000000000000597

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