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In response to the article “Ventral Longitudinal Intraspinal Fluid Collection Presenting as Upper Limb Amyotrophy” [1] we would like to point out the importance of sophisticated examinations in order to find a spinal leak in spontaneous intracranial hypotension (SIH).
In 2018, Nicoletti et al. reported on a 63-year-old man with bibrachial amyotrophy due to a spinal cerebrospinal fluid (CSF) leak, which was not located at this time [1]. Dynamic digital subtraction myelography with the patient in a prone position now showed a ventral leak at Th 6/7.
Dynamic digital subtraction myelography is the preferred imaging modality to locate a ventral CSF leak [2]. As most ventral leaks are located in the upper thoracic spine, positioning of the patient with elevation of the shoulder is important (shown in Fig. 1a–c).
Bibrachial amyotrophy is a very rare manifestation of spontaneous intracranial hypotension (SIH) and likely caused by stretching of the cervical nerve roots over the extradural CSF collection [3]. Large extradural CSF collections that may progress over the years suggest a large leak rendering the exact localization even more challenging. Minimally invasive surgery from the back of the spine revealed a distinct tear with an underlying small bony spur (shown in Fig. 1d, e).
References
Nicoletti T, Modoni A, Gaudino S, Frisullo G. Ventral Longitudinal Intraspinal Fluid Collection Presenting as Upper Limb Amyotrophy. Eur Neurol. 2018;80:126–7.
Luetzen N, Dovi-Akue P, Fung C, Beck J, Urbach H. Spontaneous intracranial hypotension: diagnostic and therapeutic workup. Neuroradiology. 2021;63:1765–72.
Schievink WI, Maya M, Moser F, Nuño M. Long-term Risks of Persistent Ventral Spinal CSF Leaks in SIH: Superficial Siderosis and Bibrachial Amyotrophy. Neurology. 2021;97:e1964–70.
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N. Lützen, A. Zeitlberger, J. Beck and H. Urbach declare that they have no competing interests.
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Lützen, N., Zeitlberger, A., Beck, J. et al. Teaching Neuroimages: Dynamic Digital Subtraction Myelography Discloses a Ventral CSF Leak in a Patient with Upper Limb Amyotrophy. Clin Neuroradiol 33, 245–246 (2023). https://doi.org/10.1007/s00062-022-01210-w
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DOI: https://doi.org/10.1007/s00062-022-01210-w