Weston-Hurst syndrome: a rare fulminant form of acute disseminated encephalomyelitis (ADEM)

Abstract

A 25-year-old Pakistani woman presented to the emergency department with a 2-day history of rapidly progressive tetraplegia followed by sudden loss of consciousness. This was preceded by an upper respiratory tract infection. On examination, she was deeply comatose with a GCS of 3/15 and intact brainstem reflexes. She was in respiratory distress, and an endotracheal tube had been passed. She had flaccid quadriplegia with depressed deep tendon reflexes and upgoing plantar response. Her MRI brain with contrast showed extensive brainstem involvement with haemorrhagic foci along with signal changes in the corpus callosum. The patient was put on a respirator. She was given IV methyl prednisolone, and later on 5 sessions of plasmapheresis were performed. After 3 months, the patient gradually recovered and started communicating. Her motor power had improved to 2/5 in her arms and 1/5 in the lower extremities. She was then discharged for further rehabilitation at home.

Brain MRI Findings in Severe COVID-19: A Retrospective Observational Study

“…The latter presentation is close to what can be observed on brain MRI scans in case of an inflammatory demyelinating disease, such as acute disseminated encephalomyelitis or acute hemorrhagic leukoencephalitis. However, these latter two diagnoses cannot only be retained on the radiologic presentation without the typical cerebrospinal fluid analysis or clinical presentation (21,22). Several putative mechanisms underlying neurologic consequences of COVID-19 are evoked, and among them are immunologic parainfectious processes (23).…”

Evolution of Neuroimaging Findings in Severe COVID-19 Patients with Initial Neurological Impairment: An Observational Study

“…Brain MRIs were considered normal, unrelated to the current acute clinical presentation, in 10 (32%) cases. Among the 21 (68%) patients with pathological brain MRIs, the neuroimaging findings were: Fourteen (45%) cases of focal (single focus or multiple foci) leptomeningeal enhancement (LME); Nine (29%) diffuse brain microhemorrhages, which predominantly involved the corpus callosum, the subtentorial juxtacortical WM, the internal capsule, the brainstem, the middle cerebellar peduncles, and the cerebellum, leading to the diagnosis of critical-illness associated cerebral microbleeds (CIAM) [ 14 ]; Four (13%) acute ischemic strokes (acute small vessel infarcts or borderline infarction); Four (13%) patients presented with arterial vessel wall thickening displaying homogeneous and concentric enhancement, compatible with cerebral vasculitis [ 5 , 6 ]; Three (10%) patients had acute inflammatory demyelinating lesions (radiological acute disseminated encephalomyelitis (ADEM) [ 15 ] or radiological acute hemorrhagic leukoencephalitis (AHL) [ 16 ]). …”

“…Three (10%) patients had acute inflammatory demyelinating lesions (radiological acute disseminated encephalomyelitis (ADEM) [ 15 ] or radiological acute hemorrhagic leukoencephalitis (AHL) [ 16 ]).…”

CASE REPORT: Fulminant acute hemorrhagic Leukoencephalitis (AHLE): A rare and ruinous outcome with cerebral herniation (COVID-19)

“…Acute hemorrhagic leukoencephalitis pathogenesis to date remains a bewildering dilemma, with up to 50% of cases have a preceding infectious (viral and bacterial) illness [ 5 ]. As observed with our patient, with progressive refractory cerebral edema post COVID-19 infection; Its speculative pathogenesis remains elusive and unraveled despite proposed underlying immune etiology similar to what is observed in ADEM, yet up to 50% of AHLE patients exhibit no preceding illnesses [ 5 , 6 ].…”