A case of Sturge–Weber syndrome with symptomatic hemiplegic migraine: Clinical and multimodality imaging data during a prolonged attack

Freilinger, Tobias; Peters, Nils; Rémi, Jan; Linn, Jennifer; Hacker, Marcus; Straube, Andreas; Pfefferkorn, Thomas

doi:10.1016/j.jns.2009.08.050

Cited by 24 publications

(22 citation statements)

References 25 publications

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“…The mechanism of migrainous attacks in SWS is suggested to be chronic focal ischemia, abnormal venous outflow, and subsequent impaired neuronal function, which might lower the threshold for cortical spreading depression and increase the susceptibility of the migrainous attacks . As for migraine with prolonged aura, there are some reports elucidating the possible pathophysiology.…”

Section: Discussionmentioning

confidence: 99%

“…Another report related to a 21‐year‐old SWS case developing a prolonged attack of hemiplegic migraine that resolved in 2 weeks. Complex changes of both cerebral and glucose metabolism contributed to the clinical manifestation . However, focal neurologic defect associated with migraine‐like events in SWS is not always benign and could become permanent.…”

Section: Discussionmentioning

confidence: 99%

“…The clinical features of SWS include epilepsy, hemiplegia, mental retardation, and glaucoma . Migraine‐like headache with transient neurological deficits including hemiplegia and hemianopsia is also a recognized manifestation . Prolong homonymous hemianopia becoming permanent visual loss without cerebral infarction is, however, rarely reported.…”

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confidence: 99%

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Type III Sturge‐Weber Syndrome With Migraine‐Like Attacks Associated With Prolonged Visual Aura

et al. 2013

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Sturge-Weber syndrome is known to be associated with migraine attacks and prolong aura even without cerebral infarction. We report the case of a 36-year-old woman with type III Sturge-Weber syndrome developing with prolonged left homonymous hemianopsia after an intractable migraine-like headache and becoming a permanent visual field defect at 18-month follow up. By adopting a multimodality imaging study, we suggested that the underlying mechanism of prolonged visual field defect was due to blood flow disturbance and vasogenic leakage under the leptomeningeal angioma combining with atrophy and the damaged integrity of white matter in right occipital lobe.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Type III Sturge‐Weber Syndrome With Migraine‐Like Attacks Associated With Prolonged Visual Aura

et al. 2013

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“…The motor weakness is often accompanied by other forms of aura, such as impaired vision, speech, or sensation. Even though the exact cause of HM is still unknown, symptomatic HM as a clinical symptom has been demonstrated to be associated with multiple neurological diseases, such as Sturge–Weber Syndrome (SWS), pulmonary arteriovenous malformation (PAVM), and moyamoya disease . However, no relation has yet to be found between HM and NIID.…”

Section: Introductionmentioning

confidence: 99%

Interrelated Pathogenesis? Neuronal Intranuclear Inclusion Disease Combining With Hemiplegic Migraine

Wang

Nie

et al. 2019

Headache

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Background.-Neuronal intranuclear inclusion disease (NIID) is considered a heterogeneous disease because of its highly variable clinical manifestations. To date, there are no reports of NIID patients presenting with hemiplegic migraine (HM)-like headache, or of HM and NIID co-occurring as comorbidity, and the connection between these 2 seemingly unrelated clinical conditions has yet to be established.Method.-We present a patient with NIID who was previously diagnosed with HM. To determine the pathogenesis of HM in this NIID patient, we systematically reviewed published NIID and HM cases and cataloged them based on their clinical manifestations.Result.-The clinical manifestations of NIID is highly various; however, there is no case reported to date that shows HMlike symptoms or cerebral edema. All documented symptomatic HM cases show vascular dysfunction to various degrees, but none of them has been shown to be correlated with NIID. Conclusion.-Our patient is the first documented case in which HM and NIID occur simultaneously. Vascular dysfunctions that cause cerebral hypoperfusion and glucose hypometabolism, two of the dominant causes of symptomatic HM, may be associated with the accumulation of eosinophilic hyaline inclusions that cause NIID. However, the existence of inclusions may also alter neuronal behavior and indirectly cause cerebral hypoperfusion and glucose hypometabolism. Further research andobservations are needed to examine the relationship between HM and NIID. Key words: hemiplegic migraine, sporadic hemiplegic migraine, neuronal intranuclear inclusion disease Abbreviations: CNS central nervous system, CSF cerebrospinal fluid, CT computed tomography, DWI diffusion-weighted imaging, EEG electroencephalography, FHM familial hemiplegic migraine, HM hemiplegic migraine, ICHD International Classification of Headache Disorders, MRA magnetic resonance angiography, MRI magnetic resonance imaging, NIID neuronal intranuclear inclusion disease, PET positron emission tomography, PWI perfusion-weighted imaging, SHM sporadic hemiplegic migraine, SWI susceptibility weighted imaging (Headache 2020;60:382-395) Headache

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“…There is no evidence that these increases are caused by ongoing seizures (since they were reported in the interictal state). Limited longitudinal data suggested that the increases in perfusion and metabolism are transient and switch to decreased perfusion and metabolism during the course of the disease [12-13, 16]. …”

Section: Introductionmentioning

confidence: 99%

A perfusion-metabolic mismatch in Sturge-Weber syndrome: A multimodality imaging study

Alkonyi

Miao

et al. 2012

Brain and Development

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OBJECTIVE We combined perfusion weighted imaging (PWI) with 2-deoxy-2[18F]fluoro-D-glucose (FDG) positron emission tomography (PET) to study the relationship between regional metabolic and perfusion abnormalities and their clinical correlates in children with Sturge-Weber syndrome (SWS). METHODS Fifteen children (age: 0.9-10 years) with unilateral SWS underwent high-resolution PWI and FDG PET prospectively. Regional (lobar) asymmetry indices (AIs) of subcortical white matter (WM) cerebral blood flow (CBF) were correlated with corresponding cortical FDG uptake asymmetries, extent of leptomeningeal vascular malformation and clinical seizure variables. RESULTS Abnormal cortical glucose metabolism and/or subcortical WM CBF were seen in all lobes affected by vascular malformation and extended to lobes not affected by abnormal pial vessels in 6 patients. Lower CBF was associated with lower cortical glucose metabolism in the temporal, parietal and occipital lobes (p≤0.02). While decreased perfusion was associated with hypometabolism in most cases, increased regional CBF (found in 6 patients) was commonly associated with relatively mild or no hypometabolism. Ten of 24 cerebral lobes with normal glucose metabolism in the affected hemisphere showed abnormal perfusion. High seizure frequency was associated with severe parieto-occipital hypoperfusion (p≤0.03), while long duration of epilepsy was related to frontal lobe hypometabolism (p=0.015). CONCLUSIONS Regional perfusion and cortical metabolic abnormalities can extend beyond lobes affected by leptomeningeal vascular malformations and are related to epilepsy in SWS. Despite a general correlation between perfusion and metabolism, increased WM perfusion with preserved cortical metabolism in overlying cortex is a common pattern of a perfusion/metabolic mismatch. This may represent a disease stage where cortical function is preserved while increased WM perfusion provides collateral drainage of cortex via the deep vein system.

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A case of Sturge–Weber syndrome with symptomatic hemiplegic migraine: Clinical and multimodality imaging data during a prolonged attack

Cited by 24 publications

References 25 publications

Type III Sturge‐Weber Syndrome With Migraine‐Like Attacks Associated With Prolonged Visual Aura

Type III Sturge‐Weber Syndrome With Migraine‐Like Attacks Associated With Prolonged Visual Aura

Interrelated Pathogenesis? Neuronal Intranuclear Inclusion Disease Combining With Hemiplegic Migraine

A perfusion-metabolic mismatch in Sturge-Weber syndrome: A multimodality imaging study

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