Locked-in syndrome in fulminant demyelinating disease.

Blunt, S B; Boulton, Jacqueline; Wise, Robert I.; Kennard, Chelsea; Lewis, P. D.

doi:10.1136/jnnp.57.4.504

Cited by 5 publications

(5 citation statements)

References 6 publications

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“…• Aneurysm [43][44][45] /vascular malformation 37,40,42,46,47 of the basilar artery • Artery dissection 10,33,[48][49][50][51] (e.g. due to cervical manipulation [52][53][54][55] • Basilar artery vasospasm 56 • Basilar migraine 57 • Central pontine myelinolysis [58][59][60] • Cocaine abuse 61 • Hyperhomocysteinemia 62 • Infection [63][64][65] mechanical brain herniation [66][67][68][69][70] • Multiple sclerosis [71][72][73] • Pontine abscess 74 • Primary 75,76 or metastatic [77][78][79] tumour • Prolonged hypoglycemia 80 • SARS-CoV-2 81,82 • Traumatic brain injury (axonal injury, 12,[83][84]…”

Section: Aetiologies Leading To Infarction/other Damage Of the Ponsmentioning

confidence: 99%

Locked-in syndrome revisited

Schnetzer

McCoy

Bergmann

et al. 2023

Ther Adv Neurol Disord

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The locked-in syndrome (LiS) is characterized by quadriplegia with preserved vertical eye and eyelid movements and retained cognitive abilities. Subcategorization, aetiologies and the anatomical foundation of LiS are discussed. The damage of different structures in the pons, mesencephalon and thalamus are attributed to symptoms of classical, complete and incomplete LiS and the locked-in plus syndrome, which is characterized by additional impairments of consciousness, making the clinical distinction to other chronic disorders of consciousness at times difficult. Other differential diagnoses are cognitive motor dissociation (CMD) and akinetic mutism. Treatment options are reviewed and an early, interdisciplinary and aggressive approach, including the provision of psychological support and coping strategies is favoured. The establishment of communication is a main goal of rehabilitation. Finally, the quality of life of LiS patients and ethical implications are considered. While patients with LiS report a high quality of life and well-being, medical professionals and caregivers have largely pessimistic perceptions. The negative view on life with LiS must be overthought and the autonomy and dignity of LiS patients prioritized. Knowledge has to be disseminated, diagnostics accelerated and technical support system development promoted. More well-designed research but also more awareness of the needs of LiS patients and their perception as individual persons is needed to enable a life with LiS that is worth living.

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Section: Aetiologies Leading To Infarction/other Damage Of the Ponsmentioning

confidence: 99%

Locked-in syndrome revisited

Schnetzer

McCoy

Bergmann

et al. 2023

Ther Adv Neurol Disord

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Section: Fulminant Ms Versus Nondisabling Msmentioning

confidence: 99%

“…In fulminant MS, the most malignant form of MS, seizures do not always occur (Blunt, Boulton, Wise, Kennard, & Lewis, 1994;Johnson, Lavin, & Whetsell, 1990;Nozaki & Abou-Fayssal, 2010;Suzuki et al, 2013;Tutuncu et al, 2011). Even in fatal cases of fulminant MS, seizures are not always observed (Blunt et al, 1994;Johnson et al, 1990;Suzuki et al, 2013). A seizure does not usually signal fulminant MS (Elenein et al, 2011;Gupta, Vasishta, Kharbanda, Vyas, & Prabhakar, 2011;Rohani & Ghourchian, 2011), and seizures do not ordinarily appear in the early days of fulminant MS (Elenein et al, 2011;Rohani & Ghourchian, 2011).…”

Section: Fulminant Ms Versus Nondisabling Msmentioning

confidence: 99%

Unprovoked seizures in multiple sclerosis: Why are they rare?

Kavčič¹,

Hofmann²

2017

Brain and Behavior

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IntroductionThe frequency of seizures in patients with multiple sclerosis (MS) ranges from 1.5% to 7.8% and is considerably more common than chance events. The etiopathogenesis of seizures in MS is still poorly understood.MethodA review of the literature on seizures and MS using PubMed.ResultsCortical gray matter involvement appears to be an all‐too‐common pathological finding in MS to play a primary role in the pathogenesis of seizures in MS patients. There is no clear relationship between seizures and the severity of MS. In approximately 10% of cases, a seizure is actually an initial neurological symptom of MS.ConclusionSearching for coherence in the occurrence of unprovoked seizures in MS directs attention to the dichotomy in MS pathology characterized by a complex intertwining of neuroinflammatory and neurodegenerative processes. The appearance (or nonappearance) of seizures in MS in relation to disease activity and disease progression indicates a distinct clinical phenotype of MS that opens up new perspectives in MS research.

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“…LiS is most commonly caused by an infarct of the ventral pontine due to occlusion or thrombosis of the basilar artery, which interrupts the corticospinal and corticobulbar fibers to the lower cranial nerves, but spares the reticular activating system. Other causes have been cited in the literature, such as tumors or encephalitis of the brainstem, traumatic lesions, central pontine myelinolysis, multiple sclerosis, and drug intoxication 3 4…”

Section: Introductionmentioning

confidence: 99%

Locked-in syndrome responding to endovascular treatment

Song

Huang

et al. 2022

J NeuroIntervent Surg

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BackgroundLocked-in syndrome (LiS) is a rare and devastating condition in patients with acute basilar artery occlusion. However, the benefits of endovascular treatment (EVT) for LiS remain unclear.ObjectiveTo assess the outcomes associated with EVT and identify the factors associated with outcomes of LiS.MethodsWe used the data of the Endovascular Treatment for Acute Basilar Artery Occlusion Study Registry (BASILAR) from 47 tertiary stroke centers in China. The included patients had LiS and received EVT or standard medical treatment (SMT) alone. The primary outcome was improvement in the modified Rankin Scale (mRS) score at 90 days.ResultsAmong the 120 patients with LiS, 92 (76.7%) received EVT and 28 (23.3%) received SMT. Compared with SMT, EVT was associated with improved mRS score (common OR (cOR)=2.68 (95% CI 1.16 to 6.20); p=0.02) and decreased mortality (aOR=0.35 (95% CI 0.13 to 0.90); p=0.03). Moreover, the benefit of EVT for LiS was sustained for at least 1 year (p=0.008). Higher baseline posterior circulation Alberta Stroke Prognosis Early CT Score (pc-ASPECTS, aOR=2.04 (95% CI 1.34 to 3.10); p<0.001) and absence of pneumonia (aOR=0.26 (95% CI 0.08 to 0.90); p=0.03) were significantly associated with favorable functional outcome at 90 days in patients who received EVT, while lower pc-ASPECTS (aOR=0.52 (95% CI 0.36 to 0.76); p<0.001) was associated with increased 90-day mortality.ConclusionsThis study found that EVT was associated with favorable functional outcomes and decreased mortality among patients with LiS. Baseline pc-ASPECTS and pneumonia were independent predictors of outcomes.

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Locked-in syndrome in fulminant demyelinating disease.

Cited by 5 publications

References 6 publications

Locked-in syndrome revisited

Locked-in syndrome revisited

Unprovoked seizures in multiple sclerosis: Why are they rare?

Locked-in syndrome responding to endovascular treatment

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