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Tumoural transformation in Van der Knaap syndrome

890 Neurology India | Volume 67 | Issue 3 | May‐June 2019 exhibiting this phenomenon. A review of literature did not reveal similar occurrences during surgery for release of tethered spinal cord.[1-4] Chowdhury et al., hypothesized a phenomenon termed as spinal‐cardiac reflex (SCR).[5] The afferent part of this reflex arc originates from direct branches of the sympathetic chain and the sinu-vertebral nerves that innervate the spinal dura mater, and the efferent part originates from the medulla oblongata, similar to the trigeminocardiac reflex arc. It may manifest as a vasovagal reaction, leading to bradycardia and/ or hypotension. Mechanical or thermal stimulation of the spinal dura, with its intrinsic as well as extrinsic innervation, may be the triggering factor for the reflex in our case, which is considered to be similar to the trigeminocardiac reflex seen during various neurosurgical procedures. Majority of such cases are of a younger age. Thus, a younger age and female gender might be the risk factors for occurrence of this phenomenon. Maintenance of an adequate depth of anesthesia is essential, and application of a local anesthetic agent to the exposed neural structures may help to reduce the incidence of this reflex. We emphasize vigilance during an otherwise simple surgical procedure such as detethering of cord, especially during traction of the dura as well as the filum terminale. Vagolytic and sympathomimetic drugs should be kept ready to treat a possible episode of acute hemodynamic disturbance.