Ventral longitudinal intraspinal fluid collection: Rare presentation as brachial amyotrophy and intracranial hypotension

Abstract

A 31 year old man presented with severe positional neck discomfort, radiating pain, progressive asymmetrical wasting and weakness of distal upper limbs. Contrast Magnetic Resonance Imaging (MRI) of the spine demonstrated a ventral extradural intraspinal fluid collection extending from upper border of C6 to lower border of T3 vertebra with pockets of dorsal collection. Three-dimensional constructive interference in steady state (CISS 3D) used in spinal imaging for identification of CSF leak corroborated with the extent seen on T2 sagittal sections; however, the site of the leak was not identified. After a year he developed disturbing postural headache which was relieved in recumbent position. Follow up MRI of brain was normal while spine demonstrated significant cervical cord atrophy and bilateral cord white matter hyperintensities. Conclusion / Clinical Relevance: We report this unusual case where local compression by VLISFC located at the cervical and upper thoracic level not only caused distal bi-brachial amyotrophy mimicking Hirayama disease but also led to secondary intracranial hypotension. An early identification and intervention could possibly have prevented the onset of ICH.

Spinal Anterior Dural Dissection: Moving From Differential to Unifying Diagnosis

“…CSF collections extending along the anterior aspect of the spinal canal have been described mostly in the context of SIH, under several names such as “spinal longitudinal extradural CSF collections” or “extra-arachnoid fluid collections.” 5,19-21 SS has been reported in association with anterior spinal CSF collections, which have been termed “ventral longitudinal intraspinal fluid collections” by the authors 10,17 but are similar on MRI to the SADDs described here. In such cases, repetitive bleeding of dilated interdural vessels is causing hemosiderin depositions in the subarachnoid space, leading to SS.…”

“…Cases of SADD revealed by BA are less frequent than those associated with intracranial hypotension or SS, with only 18 patients reported in the literature. 8,9,[11][12][13][14][15][16][17][18] However, our hypothesis is that BA is yet another clinical presentation of the same entity, as evidenced by several reports combining a motor deficit of the upper limbs and either SIH 11,14,16 or SS. 9,15 Therefore, we propose a unifying approach in which a single etiology (SADD) can be revealed by multiple clinical presentations: BA (SADD-BA), SIH (SADD-SIH), or SS (SADD-SS) (Table 3).…”

“…We found 18 cases of SADD revealed by BA published in the literature, mostly case reports and small case series 8,9,[11][12][13][14][15][16][17][18] (Table 2). Notably, all patients were men, aged between 26 and 68 years, presenting with a slowly progressing bibrachial motor deficit of the upper limbs.…”

Spontaneous Intracranial Hypotension

“…14 Bibrachial amyotrophy is a rare complication, and the underlying mechanism is compression from the ventral extradural CSF collection. 37,78 Superficial siderosis is a remote but rare complication of spontaneous intracranial hypotension, but the underlying mechanism linking spinal CSF leaks and repetitive subarachnoid hemorrhage remains unknown. 1,79 In some situations, the spinal dural defects caused by the spinal cord herniation may be the bleeding points, and this bleeding from dural lacerations may be the reason for the superficial siderosis.…”

“…Following the Monro-Kellie doctrine, cerebral venous dilation occurs during spinal CSF leaks, resulting in slowing cerebral venous flow velocity, which causes a hypercoagulable state and venous thrombosis 14 . Bibrachial amyotrophy is a rare complication, and the underlying mechanism is compression from the ventral extradural CSF collection 37,78 . Superficial siderosis is a remote but rare complication of spontaneous intracranial hypotension, but the underlying mechanism linking spinal CSF leaks and repetitive subarachnoid hemorrhage remains unknown 1,79 .…”