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October 25, 2022; 99 (17) Resident & Fellow Section

Teaching Video NeuroImage: Spasmus Nutans, an Infantile Nystagmus

Tommaso Bellini, Marta Ferretti, Stefania Santaniello, Federico Pezzotta, Emanuela Piccotti, Pasquale Striano
First published August 26, 2022, DOI: https://doi.org/10.1212/WNL.0000000000201174
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A 5-month-old boy came to the emergency department because of a monocular nystagmus with head-nodding and torticollis (Video 1). Fundoscopic examination excluded macular/optic nerve hypoplasia and retinal abnormalities. Brain MRI was unremarkable (Figure). A diagnosis of spasmus nutans (SN) was made.

Video 1

Video shows a high-frequency, small-amplitude, intermittent, horizontal right nystagmus (part 1) accompanied by HN (part 2) that is believed to be compensatory and may evoke the vestibular ocular reflex that may dampen the SN; at sixth second, the patient presents brief binocular nystagmus before its resolution.Download Supplementary Video 1 via http://dx.doi.org/10.1212/201174_Video_1

Figure
Figure Brain MRI

(A) T2 sequence axial plane shows no lesions in optic nerves (red arrows) and a right parietal positional plagiocephaly related to torticollis. (B) T2 sequence axial plane shows no lesions in optic chiasm. (C) T2 sequence coronal plane at the blue line.

SN is a monocular/bilateral horizontal nystagmus with head-nodding and torticollis1; it usually disappears within 2 years but can persist until 12 years with an increased risk of developmental delay.

Ophthalmologic evaluation and MRI are mandatory to confirm the diagnosis and to distinguish SN from SN-like diseases with underlying retinal, optic chiasm, or brain lesions.2

Study Funding

No targeted funding reported.

Disclosure

The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.

Appendix Authors

Table

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Submitted and externally peer reviewed. The handling editor was Whitley Aamodt, MD, MPH.

  • Teaching slides links.lww.com/WNL/C277

  • Received March 1, 2022.
  • Accepted in final form July 14, 2022.

References

  1. 1.↵
    1. Gottlob I,
    2. Zubcov AA,
    3. Wizov SS,
    4. Reinecke RD
    . Head nodding is compensatory in spasmus nutans. Ophthalmology. 1992;99(7):1024-1031. doi:10.1016/s0161-6420(92)31855-x.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Bowen M,
    2. Peragallo JH,
    3. Kralik SF,
    4. Poretti A,
    5. Huisman TAGM,
    6. Soares BP
    . Magnetic resonance imaging findings in children with spasmus nutans. J AAPOS. 2017;21(2):127-130. doi:10.1016/j.jaapos.2017.03.001.
    OpenUrlCrossRef

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