Management of cervical CSF-venous fistula causing acute cognitive impairment and coma.

CSF-venous fistulas (CVFs) are increasingly recognised as a cause of spontaneous intracranial hypotension. They may present atypically including with brain sagging pseudo-dementia. Cervical CVFs are rare and their management can be difficult due to associated eloquent nerve roots. We report the case of a 49-year-old woman who presented with cognitive decline progressing to coma. Brain imaging showed features of spontaneous intracranial hypotension and a right C7 CVF was identified at digital subtraction and CT myelography. Initial treatment with CT-guided injection of fibrin sealant produced temporary improvement in symptoms before surgical treatment resulted in total clinical remission and radiological resolution.

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.

IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma.

Hypertrophic pachymeningitis is a rare disorder of the dura mater of the spine or brain. It can be caused by inflammatory, infective or neoplastic conditions or can be idiopathic. We report a man with hypertrophic pachymeningitis and bilateral chronic subdural haematoma caused by IgG4-related disease. We highlight the diagnostic challenges and discuss possible underlying mechanisms of subdural haematoma formation in inflammatory conditions. Isolated IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma is very rare; previously reported cases have suggested a possible predilection for men in their sixth decade, presenting with headache as the dominant symptom. Given the rarity and complexity of the condition, it should be managed in a multidisciplinary team setting.

"Flow void sign": flow artefact on T2-weighted MRI can be an indicator of dural defect location in ventral type 1 spinal CSF leaks.

Patients with spontaneous intracranial hypotension caused by type 1 dural defects typically have an epidural fluid collection on MRI, but the location of the defect is not usually readily identifiable on standard MRI sequences and can be at any point along the length of the collection. The most common location for type 1 leaks is ventral and as such are most commonly associated with ventral predominant epidural fluid. Dynamic myelography (either digital subtraction myelography or dynamic CT myelography) is currently the standard of care to localise the defect. We describe an imaging sign on T2-weighted images caused by CSF-flow egress at the site of the defect that may permit accurate prediction of the site of the CSF leak non-invasively. Importantly, this sign was only observed on 2D T2-weighted and STIR images and not on 3D acquisitions, which notably suppress artefact. This has implications for optimal MRI spine protocol construction. This sign can be used to limit myelographic range, reduce radiation dose and may increase diagnostic confidence to dural defect location.ABBREVIATIONS: dCTM = Dynamic CT Myelography; DSM = Digital Subtraction Myelography; SIH = Spontaneous Intracranial Hypotension.

Systemic Inflammation Is Associated With Neurologic Involvement in Pediatric Inflammatory Multisystem Syndrome Associated With SARS-CoV-2.

OBJECTIVE: Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a severe immune-mediated disorder. We aim to report the neurologic features of children with PIMS-TS. METHODS: We identified children presenting to a large children's hospital with PIMS-TS from March to June 2020 and performed a retrospective medical note review, identifying clinical and investigative features alongside short-term outcome of children presenting with neurologic symptoms. RESULTS: Seventy-five patients with PIMS-TS were identified, 9 (12%) had neurologic involvement: altered conciseness (3), behavioral changes (3), focal neurology deficits (2), persistent headaches (2), hallucinations (2), excessive sleepiness (1), and new-onset focal seizures (1). Four patients had cranial images abnormalities. At 3-month follow-up, 1 child had died, 1 had hemiparesis, 3 had behavioral changes, and 4 completely recovered. Systemic inflammatory and prothrombotic markers were higher in patients with neurologic involvement (mean highest CRP 267 vs 202 mg/L, p = 0.05; procalcitonin 30.65 vs 13.11 µg/L, p = 0.04; fibrinogen 7.04 vs 6.17 g/L, p = 0.07; d-dimers 19.68 vs 7.35 mg/L, p = 0.005). Among patients with neurologic involvement, these markers were higher in those without full recovery at 3 months (ferritin 2284 vs 283 µg/L, p = 0.05; d-dimers 30.34 vs 6.37 mg/L, p = 0.04). Patients with and without neurologic involvement shared similar risk factors for PIMS-TS (Black, Asian and Minority Ethnic ethnicity 78% vs 70%, obese/overweight 56% vs 42%). CONCLUSIONS: Broad neurologic features were found in 12% patients with PIMS-TS. By 3-month follow-up, half of these surviving children had recovered fully without neurologic impairment. Significantly higher systemic inflammatory markers were identified in children with neurologic involvement and in those who had not recovered fully.

Cerebral Venous Sinus Thrombosis, a Nonenhanced CT Diagnosis?

Purpose. Retrospectively evaluate the density of cerebral venous sinuses in nonenhanced head CTs (NCTs) and correlate these with the presence or absence of a cerebral venous sinus thrombus (CVST). Materials and Methods. Institutional review board approval was obtained and informed consent waived prior to commencing this retrospective study. Over a two-year period, all CT venograms (CTVs) performed at our institution were retrieved and the preceding/subsequent NCTs evaluated. Hounsfield Units (HUs) of thrombus when present as well as that of normal superior sagittal and sigmoid sinuses were measured. HU of thrombus was compared to that of normal vessels with and without standardisation to the average HU of the internal carotid arteries. Results. 299 CTVs were retrieved, 26 with a thrombus. Both raw and standardised HU measurements were significantly higher in CVST (p < 0.0001) compared to normal vessels. Both raw and standardised HUs are good predictors of CVST. A HU of ≥67 and a standardised measurement of ≥1.5 are associated with high probability of CVST on NCT. Conclusion. Cerebral venous sinus HU measurements may help improve sensitivity and specificity of NCT for venous sinus thrombosis and avoid potentially unnecessary follow-up examinations.