Ruptured vertebrobasilar junction aneurysm unmasking subclavian steal syndrome.

BACKGROUND: Subclavian steal occurs due to stenosis or occlusion of the subclavian artery or innominate artery proximal to the origin of the vertebral artery. Often asymptomatic, the condition may be unmasked due to symptoms of vertebrobasilar insufficiency triggered by strenuous physical exercise involving the affected upper limb. The association of vertebrobasilar junction (VBJ) aneurysms with subclavian steal syndrome has been rarely reported. Hereby, we present a case of VBJ aneurysm associated with subclavian steal treated successfully with endovascular coiling. CASE DESCRIPTION: A 65-year-old female presented in the emergency department with acute severe headache and vomiting with no focal neurological deficits. Non-contrast computed tomography of the brain showed modified Fischer Grade 3 subarachnoid hemorrhage. Subsequent digital subtraction angiogram (DSA) showed VBJ aneurysm directed inferiorly with the left subclavian artery occlusion. There was retrograde filling of the left vertebral artery on right vertebral injection, confirming the diagnosis of subclavian steal. Balloon assisted coiling of the VBJ aneurysm was performed while gaining access through the stenotic left vertebral artery ostium which provided a more favorable hemodynamic stability to the coil mass. CONCLUSION: Subclavian steal exerting undue hemodynamic stress on vertebrobasilar circulation can be an etiological factor for the development of the flow-related aneurysms. Access to the VBJ aneurysms may be feasible through the stenosed vertebral artery if angioplasty is performed before the coiling of the aneurysm.

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

CONTEXT: Ventral longitudinal intraspinal fluid collection (VLISFC) presenting as hand amyotrophy has been described only in a few cases and there are no reports on associated intracranial CSF hypovolemia (ICH). We describe the clinical and imaging findings of a case with combined brachial amyotrophy and ICH secondary to VLISFC. FINDINGS: 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.

Monomelic amyotrophy: clinical profile and natural history of 279 cases seen over 35 years (1976-2010).

Our objective was to study the clinical characteristics and natural history of monomelic amyotrophy (MMA). We used a retrospective study of 279 patients diagnosed to have either upper (Hirayama disease) or lower limb MMA. Results showed that brachial MMA (BMMA) occurred in 224 patients (male:female, 9:1). Mean age of onset was 19.5 ± 4.18 years. Progression occurred over less than five years in the majority (95.9%) of patients. Duration at the last follow-up was: up to five years in 61.4%, 5-10 in 21.3%, 10-15 in 7.2%, > 15 years in 10.1%. MRI showed asymmetrical lower cervical cord atrophy in 44.6% of patients. Crural MMA (CMMA) occurred in 55 patients (male:female, 13:1). Mean age of onset was 21.38 ± 5.3 years. Similar to BMMA, most cases (65.5%) had onset between 15 and 25 years of age. Total duration of illness at the last follow-up was up to five years in 52.7%, 10 and beyond in 47.3%. In conclusion, a large cohort of patients with monomelic amyotrophy seen over 35 years (1976-2010) is described. Study data support the clinical findings and its natural history with long term follow-up, and the findings emphasize that monomelic amyotrophy is a 'benign' condition with a self-limiting course.