Neuropsychological and neuroimaging characteristics of classical superficial siderosis

Chan, Edgar; Sammaraiee, Yezen; Banerjee, Gargi; Martin, Andreas Flores; Farmer, Simon F.; Cowley, Peter; Sayal, Parag; Kharytaniuk, Natallia; Eleftheriou, Perla; Porter, John B.; Harskamp, Natasja van; Cipolotti, Lisa; Werring, David J.

doi:10.1007/s00415-021-10548-z

Cited by 12 publications

(28 citation statements)

References 27 publications

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“…No viral RNA was detected in the CSF of the patients sampled, which is consistent with the published literature [ 10 ]. While this suggests no direct neuronal damage exerted by viral replication within the CNS in these patients, other inflammatory mechanisms may be implicated.…”

Section: Discussionsupporting

confidence: 91%

“…The latency of myoclonus after onset of COVID symptoms was a mean of 22.6 days (interquartile range, IQR 19.5–26 days) in this series. This is consistent with an autoinflammatory mechanism and marginally higher than the latency in the cases published to date, which is 13 days (IQR 11–21 days) [ 10 ]. This may be explained by the heavy sedation and use of neuromuscular blockade in this series, which might have caused a delay in recognition of motor symptoms.…”

Section: Discussionsupporting

confidence: 84%

“…Several cases and case series describing COVID-19 patients with myoclonus have been published to date [ 10 ]. The severity of respiratory disease was variable and ranged from asymptomatic cases only diagnosed via serology [ 11 ] to critically ill patients requiring mechanical ventilation [ 12 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…The severity of respiratory disease was variable and ranged from asymptomatic cases only diagnosed via serology [ 11 ] to critically ill patients requiring mechanical ventilation [ 12 , 13 ]. A literature review found that 53.3% of patients with myoclonus and COVID-19 required ICU admission [ 10 ]. This suggests that hypoxia, among other components of toxic metabolic encephalopathy, might be a contributing factor in the pathogenesis of myoclonus [ 14 ]; however, considering the number of cases without pulmonary involvement [ 10 ], additional mechanisms must be considered.…”

Section: Discussionmentioning

confidence: 99%

“…A literature review found that 53.3% of patients with myoclonus and COVID-19 required ICU admission [ 10 ]. This suggests that hypoxia, among other components of toxic metabolic encephalopathy, might be a contributing factor in the pathogenesis of myoclonus [ 14 ]; however, considering the number of cases without pulmonary involvement [ 10 ], additional mechanisms must be considered.…”

Section: Discussionmentioning

confidence: 99%

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COVID-19-associated myoclonus in a series of five critically ill patients

Grieb¹,

Seitz²,

Kitzberger³

et al. 2021

Wien Klin Wochenschr

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Summary Background In addition to respiratory symptoms, many patients with coronavirus disease 2019 (COVID-19) present with neurological complications. Several case reports and small case series described myoclonus in five patients suffering from the disease. The purpose of this article is to report on five critically ill patients with COVID-19-associated myoclonus. Material and methods The clinical courses and test results of patients treated in the study center ICU and those of partner hospitals are described. Imaging, laboratory tests and electrophysiological test results are reviewed and discussed. Results In severe cases of COVID-19 myoclonus can manifest about 3 weeks after initial onset of symptoms. Sedation is sometimes effective for symptom control but impedes respiratory weaning. No viral particles or structural lesions explaining this phenomenon were found in this cohort. Conclusion Myoclonus in patients with severe COVID-19 may be due to an inflammatory process, hypoxia or GABAergic impairment. Most patients received treatment with antiepileptic or anti-inflammatory agents and improved clinically.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

COVID-19-associated myoclonus in a series of five critically ill patients

Grieb¹,

Seitz²,

Kitzberger³

et al. 2021

Wien Klin Wochenschr

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Progressive loss of hearing and balance in superficial siderosis due to occult spinal dural defects

Hálmagyi

Parker

Chen

et al. 2022

Eur Arch Otorhinolaryngol

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Purpose Superficial siderosis, a progressive, debilitating, neurological disease, often presents with bilateral impairment of auditory and vestibular function. We highlight that superficial siderosis is often due to a repairable spinal dural defect of the type that can also cause spontaneous intracranial hypotension. Methods Retrospective chart review of five patients presenting with moderate to severe, progressive bilateral sensorineural hearing loss as well as vestibular loss. All patients had developed superficial siderosis from spinal dural defects: three after trauma, one after spinal surgery and one from a thoracic discogenic microspur. Results The diagnosis was made late in all five patients; despite surgical repair in four, hearing and vestibular loss failed to improve. Conclusions In patients presenting with progressive bilateral sensorineural hearing loss, superficial siderosis should be considered as a possible cause. If these patients also have bilateral vestibular loss, cerebellar impairment and anosmia, then the diagnosis is likely and the inevitable disease progress might be halted by finding and repairing the spinal dural defect.

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