Meling Torstein R, Nouri Aria, May Adrien, Guinand Nils, Vargas Maria Isabel, Destrieux Christophe
Department of Neurosurgery, Geneva University Hospitals, University of Geneva, Rue Gabrielle-Perret-Gentil 4, 1205, Geneva, Switzerland.
Faculty of Medicine, University of Geneva, Geneva, Switzerland.
J Neurol. 2020 Oct;267(10):2865-2870. doi: 10.1007/s00415-020-09891-4. Epub 2020 May 26.
CNS cavernomas are a type of raspberry-shaped vascular malformations that are typically asymptomatic, but can result in haemorrhage, neurological injury, and seizures. Here, we present a rare case of a brainstem cavernoma that was surgically resected whereafter an upbeat nystagmus presented postoperatively.
A 42-year old man presented with sudden-onset nausea, vomiting, vertigo, blurred vision, marked imbalance and difficulty swallowing. Neurological evaluation showed bilateral ataxia, generalized hyperreflexia with left-sided predominance, predominantly horizontal gaze evoked nystagmus on right and left gaze, slight left labial asymmetry, uvula deviation to the right, and tongue deviation to the left. MRI demonstrated a 13-mm cavernoma with haemorrhage and oedema in the medulla oblongata. Surgery was performed via a minimal-invasive, midline approach. Complete excision was confirmed on postoperative MRI. The patient recovered well and became almost neurologically intact. However, he complained of mainly vertical oscillopsia. The videonystagmography revealed a new-onset spontaneous upbeat nystagmus in all gaze directions, not suppressed by fixation. An injury of the rarely described intercalatus nucleus/nucleus of Roller is thought to be the cause.
Upbeat nystagmus can be related to several lesions of the brainstem, including the medial longitudinal fasciculus, the pons, and the dorsal medulla. To our knowledge, this is the first case of an iatrogenic lesion of the nucleus intercalatus/nucleus of Roller resulting in an upbeat vertical nystagmus. For neurologists, it is important to be aware of the function of this nucleus for assessment of clinical manifestations due to lesions within this region.
中枢神经系统海绵状血管瘤是一种覆盆子状血管畸形，通常无症状，但可导致出血、神经损伤和癫痫发作。在此，我们报告一例罕见的脑干海绵状血管瘤病例，该病例接受了手术切除，术后出现了向上性眼球震颤。
一名42岁男性突发恶心、呕吐、眩晕、视力模糊、明显失衡和吞咽困难。神经学评估显示双侧共济失调、全身反射亢进且左侧更明显、左右注视时主要为水平性凝视诱发性眼球震颤、左侧唇部轻度不对称、悬雍垂偏向右侧、舌头偏向左侧。磁共振成像（MRI）显示延髓有一个13毫米的伴有出血和水肿的海绵状血管瘤。通过微创中线入路进行了手术。术后MRI证实完全切除。患者恢复良好，神经功能几乎完全恢复。然而，他主要抱怨垂直性视振荡。眼震电图显示在所有注视方向均出现新发的自发性向上性眼球震颤，不被注视抑制。据认为，很少被描述的中间层核/罗勒核损伤是病因。
向上性眼球震颤可与脑干的多种病变相关，包括内侧纵束、脑桥和延髓背侧。据我们所知，这是首例因中间层核/罗勒核医源性损伤导致向上性垂直性眼球震颤的病例。对于神经科医生而言，了解该核的功能对于评估该区域病变所致的临床表现很重要。
