Weston-Hurst syndrome, how to recognize - a case report

In this report we present a 27-year-old female patient with acute tetraparesis, diffuse paresthesias and drowsiness five days after generalized myalgia, cough and fatigue. Within a day she became comatose and was transferred to the intensive care unit. Brain and spinal cord MRI revealed multifocal white matter demyelinating lesions and longitudinally extensive myelitis associated with hemorrhagic lesions in both brainstem and spinal cord. Extensive investigation was negative, including AQP4, MOG and antineuronal antibodies panel. Treatment was performed with methylprednisolone, plasmapheresis, immunoglobulin and cyclophosphamide with partial recovery. After 118 days, the patient was discharged with quadriplegia with discret improvement of the upper limbs and preserved consciousness. Not only the clinical presentation, with rapidly progressive and severe course, but also the MRIs were compatible with hemorrhagic acute disseminated encephalomyelitis (ADEM), also known as Weston-Hurst syndrome. Hemorrhagic ADEM is considered as a variant of ADEM, most commonly seen in adults, in contrast to its prototype disease. Up to this day, the etiology is still not comprehended, however it is hypothesised to be an autoimmune process associated with viral or bacterial infection causing molecular mimicry. Considering the high mortality (up to 70%), early diagnosis and referral to specialized centers are crucial for appropriate therapy, based on immunossupression.