Spinomedullary Weston Hurst Syndrome After COVID-19 and Influenza Co-Infection: A Case Report

Abstract

The neurological complications of coronavirus disease 2019 (SARS-CoV-2, COVID-19) have so far included a range of para- and post-infectious neuroinflammatory syndromes inclusive of all components of the neuraxis and peripheral neuromuscular system. In comparison to the para-infectious manifestations of anosmia, ageusia, encephalopathy, and encephalitis, cases of post-infectious ADEM have rarely been reported and have most commonly affected the supratentorial component with or without spinal cord involvement. In this report, we describe a case of isolated involvement of the cervicothoracic spinal cord and medulla, occurring in association with microhemorrhages and hemosiderin deposition in the medulla, that presented fulminantly and required aggressive immunotherapy to control the inflammatory attack. We compare and contrast this case against prior reports of acute hemorrhagic leukoencephalitis (Weston Hurst syndrome) and review the atypical features of neuroinflammation reported to occur following COVID-19 infection.

Response to Letter to the Editor: Spinomedullary Weston Hurst Syndrome After COVID-19 and Influenza Co-Infection

“…For this very reason, we were careful to highlight in Tables 1 and 2 the extensive laboratory testing of serum and CSF specimens that occurred in this case, including all of the potential alternative diagnoses suggested in the letter. 1 In the initial phase of the diagnostic work-up, we were equally concerned about the possibility for sarcoidosis and malignancy (lymphoma included) as well as non-inflammatory myelopathies (including vascular myelopathies, such spinal cord infarction and dural arteriovenous fistulas), which sparked our decision to obtain whole body PET CT and MRA of spinal blood vessels, both of which were unrevealing of abnormalities to suggest an alternative diagnosis. CLIPPERS most commonly manifests radiographically as punctate or curvilinear areas of enhancement centered predominantly in the pons and cerebellum; the large lesion in the medulla of our patient with confluent longitudinal extension into the spinal cord would be very atypical for this disorder (as illustrated in Figure 1).…”

“…CLIPPERS most commonly manifests radiographically as punctate or curvilinear areas of enhancement centered predominantly in the pons and cerebellum; the large lesion in the medulla of our patient with confluent longitudinal extension into the spinal cord would be very atypical for this disorder (as illustrated in Figure 1). 1,2 With such extensive testing conducted, we believe that alternative etiologies were thoroughly investigated and appropriately excluded as outlined in the original report.…”

“…The patient's quadriplegia was accompanied by other exam findings of upper motor neuron dysfunction, including spasticity and hyperreflexia, clinically in keeping with a lesion of the central nervous system that was subsequently identified on MRI as displayed in Figure 1. 1 The episode of altered consciousness was presumed to be a seizure and managed with levetiracetam. EEG monitoring subsequent to the event only revealed generalized slowing without epileptiform abnormalities or seizures.…”

Rule Out Alternative Causes Before Attributing Acute, Haemorrhagic, Leukoencephalitis (AHLE) to COVID-19

“…We read with interest the article by Hutto et al about a 22 years old male who developed rapidly sequential headache, numbness of both arms, gait disturbance, and respiratory insufficiency requiring mechanical ventilation, 2 months after a compound infection with SARS-CoV-2 and influenza A 1 . COVID-19 had manifested with ageusia, anosmia, myalgia, and dyspnoea and had completely resolved without sequelae after 1 week under outpatient management 1 .…”

“…We read with interest the article by Hutto et al about a 22 years old male who developed rapidly sequential headache, numbness of both arms, gait disturbance, and respiratory insufficiency requiring mechanical ventilation, 2 months after a compound infection with SARS-CoV-2 and influenza A 1 . COVID-19 had manifested with ageusia, anosmia, myalgia, and dyspnoea and had completely resolved without sequelae after 1 week under outpatient management 1 . Neurological work-up revealed spino-medullary acute, haemorrhagic leukoencephalitis (AHLE, Weston Hurst syndrome) which partially resolved upon administration of steroids, plasmapheresis, cyclophosphamide, and intravenous immunoglobulins (IVIG) 1 .…”