Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension

BACKGROUND: We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG). METHODS: A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH. RESULTS: SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided. CONCLUSIONS: This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH

Rupture of a Previously Documented Small Asymptomatic Intracranial Aneurysm in a Patient With Autosomal Dominant Polycystic Kidney Disease

Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography

Surgical of Superficial Siderosis Associated With a Treatment Spinal Arteriovenous Malformation

In many patients with superficial siderosis of the central nervous system (CNS) no source of bleeding can be established, despite extensive examinations. The authors report a patient with superficial siderosis and a spinal arteriovenous malformation (AVM) that was not visible on magnetic resonance (MR) imaging or myelography but was identified on angiographic studies. This 71-year-old man presented with a 2-year history of progressive gait difficulties and heating loss. Examination showed ataxia, hearing loss, and quadriparesis. On MR imaging superficial siderosis of the brain and spinal cord as seen; however, MR imaging of the CNS, as well as cerebral angiography and myelography studies, did not reveal the source of hemorrhage. Spinal angiography revealed a small slow-flow pial AVM at the C-5 level originating from the anterior spinal artery. A C-5 corpectomy was performed and the AVM was obliterated. The patient did well and reported no further progression of his symptoms during 3 months of follow up. Spinal angiography is indicated to complete the evaluation of patients with superficial siderosis, even if results of spinal MR imaging and myelography studies are normal. Obliteration of spinal AVMs may successfully prevent the progression of superficial siderosis