Sturge-Weber syndrome with late onset hemiplegic migraine-like attacks and progressive unilateral cerebral atrophy

Planche, Vincent; Chassin, Olivier; LeDuc, Lawrence; Regnier, Wendy; Kelly, A.; Colamarino, R.

doi:10.1177/0333102413505237

Cited by 21 publications

(22 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…We identified 7 cases of symptomatic HM: 3 patients with SWS and 1 patient with Moyamoya disease, CREST syndrome, PAVM, and systemic lupus erythematosus (SLE; Table ) . Each of the diseases shows evidence of vascular dysfunction, including both systemic and CNS vascular disorders.…”

Section: Resultsmentioning

confidence: 99%

“…As we perform our literature review regarding symptomatic HM, we find that vascular‐related theory is more substantiated by cases. (See Tables and ) Diseases including SWS, CREST syndrome, PAVM, Moyamoya disease, and SLE are prevalent causes of HM. Although these patients had no characteristic age at onset, sex, headache side, headache duration, weakness side or weakness duration, and frequency, they all show vascular abnormalities to various degrees, including both central and systemic blood vessels.…”

Section: Discussionmentioning

confidence: 99%

“…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%

See 2 more Smart Citations

Interrelated Pathogenesis? Neuronal Intranuclear Inclusion Disease Combining With Hemiplegic Migraine

Wang

Nie

et al. 2019

Headache

View full text Add to dashboard Cite

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

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Interrelated Pathogenesis? Neuronal Intranuclear Inclusion Disease Combining With Hemiplegic Migraine

Wang

Nie

et al. 2019

Headache

View full text Add to dashboard Cite

show abstract

“…20 Although it is challenging to appreciate the complete FDG-PET findings without a thorough analysis of the data, it appears that the axial map provided in Fig 2A of that report could suggest marked hypometabolism associated with the DVA in the right cerebellum relative to the homologous contralateral region. In another case report, Planche et al 21 described a patient with a DVA and cortical hypometabolism demonstrated on FDG-PET, though this finding was confounded by superimposed pathology of Sturge-Weber syndrome, including extensive pial angiomatosis. Brain metabolism has been previously evaluated by using FDG-PET in patients with cavernous malformations.…”

Section: Figmentioning

confidence: 99%

Brain Metabolic Abnormalities Associated with Developmental Venous Anomalies

Larvie

Timerman

Thum

2014

AJNR Am J Neuroradiol

View full text Add to dashboard Cite

show abstract

“…In one case of Sturge‐Weber syndrome who had refractory headache associated with aura‐like manifestations, a remarkable improvement was reported under LTG at 25 mg/day . In another individual, escalation of LTG dose up to 200 mg/day prevented the occurrence of repeated and long‐lasting hemiplegic aura‐like manifestations . These manifestations with a long progression of aura symptoms appear clearly distinct from ictal headache commonly misdiagnosed as MA .…”

Section: Accumulating Evidence Suggests That Lamotrigine May Reduce Tmentioning

confidence: 97%

Lamotrigine in the Prevention of Migraine With Aura: A Narrative Review

Buch

Chabriat

2019

Headache

View full text Add to dashboard Cite

Background Lamotrigine is not recommended in the prevention of migraine in general but some reports suggest that it might be effective for treating specifically migraine with aura (MA). This review aims to summarize the related data from the literature and to better understand this discrepancy. Methods All reports from the literature related to the use of lamotrigine in migraine with or without aura published prior to February 2019 found using PUBMED and the 2 keywords “migraine” AND “lamotrigine” were reviewed. Original studies, published in full, systematic reviews, and all case reports were synthetized. We also examined the risk profile, pharmacokinetics, and mode of action of lamotrigine in view of the presumed mechanism of MA. Results Lamotrigine was tested in different populations of migraineurs, but previous studies had small sample sizes (n < 35) and might not have been powered enough for detecting a potential benefit of lamotrigine in MA. Accumulating data suggest that the drug can reduce both the frequency and severity of aura symptoms in multiple conditions and is well tolerated. Conclusion Lamotrigine appears promising for treating attacks of MA and related clinical manifestations because of its high potential of efficacy, low‐risk profile, and cost. Additional studies are needed for testing lamotrigine in patients with MA.

show abstract

Sturge-Weber syndrome with late onset hemiplegic migraine-like attacks and progressive unilateral cerebral atrophy

Abstract: We propose to consider SWS as a cause of apparently isolated hemiplegic migraine and lamotrigine as a preventive medication in HM-like attacks.

Cited by 21 publications

References 12 publications

Interrelated Pathogenesis? Neuronal Intranuclear Inclusion Disease Combining With Hemiplegic Migraine

Interrelated Pathogenesis? Neuronal Intranuclear Inclusion Disease Combining With Hemiplegic Migraine

Brain Metabolic Abnormalities Associated with Developmental Venous Anomalies

Lamotrigine in the Prevention of Migraine With Aura: A Narrative Review

Contact Info

Product

Resources

About