Background: Cortical demyelination and meningeal inflammation have been detected neuropathologically in multiple sclerosis (MS) and recently in myelin oligodendrocyte glycoprotein antibody disease (MOGAD). Objectives: To assess in vivo cortical and leptomeningeal involvement in MOGAD. Methods: We prospectively evaluated 11 MOGAD and 12 relapsing-remitting MS (RRMS) patients combining three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) and 3D-T1-weighted (3D-T1w) sequences at 3-Tesla magnetic resonance imaging (MRI). Leptomeningeal contrast enhancement (LMCE) was assessed on 3D-FLAIR post-gadolinium (3D-FLAIRGd). Cerebral cortical lesions (CCLs) were classified as either intracortical–subpial (IC–SP) or leukocortical (LC). Results: CCLs were present in 8/11 MOGAD and 12/12 RRMS patients, with the number of CCLs being significantly lower in MOGAD (median (interquartile range (IQR)) 3 (0.5–4) vs 12 (4.75–19), p = 0.0032). In MOGAD, IC–SP lesions were slightly more prevalent than LC lesions (2 (0–2.5) vs 1 (0–2), p = 0.6579); whereas in RRMS, IC–SP lesions were less prevalent than LC lesions (3.5 (2.75–5.5) vs 9 (2–12.75), p = 0.27). LMCE was observed in 3/11 MOGAD and 1/12 RRMS patients; MOGAD with LMCE showed an increased median number of CCLs compared with MOGAD without LMCE (8 (4–9) vs 2.5 (0.75–3.25), p = 0.34). No correlation was observed between MOGAD MRI findings and (a) MOGAD duration, (b) serum MOG-immunoglobulin G1 titers, and (c) oligoclonal band presence. Conclusion: We described cortical lesion topography and detected for the first time LMCE using 3D-FLAIRGd sequences in MOGAD patients.
Background
Spontaneous intracranial hypotension (SIH) is characterized by positional headache caused by low CSF pressure, without any major traumatic event. Optimal treatment is still debated; epidural blood patch (EBP) is usually used after unsuccessful conservative treatment with variable efficacy and potentially severe complications. Although steroids have been reported to be beneficial, their effectiveness is still controversial, and more clinical evidence is needed.
Case presentation
A 37-year-old woman was admitted to the neurology department due to severe orthostatic headache with nausea over the last 5 days. No trauma history or spinal manipulation were mentioned. On arrival, neurological examination, brain CT, and laboratory investigation were normal. Intracranial hypotension was clinically suspected, and lumbar puncture revealed low opening pressure. Brain MRI demonstrated pachymeningeal gadolinium enhancement and distended and rounded dural venous sinuses, while cervicothoracic spine MRI revealed thoracic CSF leakage, leading to SIH diagnosis. The patient was treated with high-dose intravenous methylprednisolone, with complete clinical resolution within 24 h.
Conclusions
Our case, combined with literature evidence, supports the high-dose intravenous corticosteroids as a reasonable treatment option in selected cases, before trying EBP or surgical repair. Randomized clinical trials are needed, in order to optimize SIH patients’ outcomes.
Bilateral lesions of medial cerebellar peduncles are rare and are most frequently observed in acute cerebral infarction due to large-artery atherosclerosis, wallerian degeneration, neurodegenerative diseases (i.e. multiple system atrophy), inflammatory diseases (i.e. neuromyelitis optica, acute demyelinating encephalomyelitis), toxic encephalopathies (i.e. heroin abuse) and primary central nervous system lymphomas [3]. Wallerian degeneration regards progressive anterograde demyelination of the distal axons following injury to the proximal axon. As a result, fibrosis and atrophy of the affected fiber tracts occurs [2]. We present a case report of a patient with the rare finding of bilateral middle cerebral peduncle lesions, due to wallerian degeneration following pontine infarction. We further discuss differential diagnosis upon imaging findings and their clinical utility.
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