RESUMO
Background: Acute hemorrhagic leukoencephalitis (AHLE) is a very rare demyelinating disease with rapid fulminant inflammation of the white matter. Although the exact etiology is unknown, AHLE usually manifests post a viral or bacterial infection and less often seen post vaccination for measles or rabies. AHLE has a very poor prognosis and a high mortality rate. Owing to the rarity of this entity there is not clear consensus on the proper line of management. In this report, we present a case of AHLE as a para-infectious sequel to COVID-19 in a young patient. Clinical presentation: We report a 30-year-old turkish patient with an initial presentation of upper respiratory tract infection due to COVID-19. Initially, she was admitted to the hospital with generalized tonic-clonic seizure (GTCS) and deterioration in her level of consciousness lapsing into a coma. An initial CT scan showed diffuse brain edema and an MRI head confirmed the suspicion of Acute hemorrhagic leukoencephalitis (AHLE). Despite prompt and diligent osmotic therapy and pulsed intravenous (IV) methylprednisolone, her condition rapidly depreciated and progressed into cerebral edema with gravid sequela of brainstem herniation. Conclusions: AHLE is a very rare entity and perhaps its fulminant debilitating course and high mortality should warrant further studies on disease pathophysiology and its optimal treatment parameters. Life-saving decompressive hemicraniectomy should be considered in the multidisciplinary approach of the management with tailored osmotic and immunotherapy.
RESUMO
BACKGROUND: Polymyositis is a rare medical disorder complicating pregnancy. Ventilatory muscle weakness leading to respiratory failure is an uncommon manifestation of this autoimmune disease. We report a case of life-threatening hypercapnic respiratory failure due to polymyositis-related respiratory muscle weakness in a pregnant woman. CASE PRESENTATION: A 31-year-old, African woman in her second trimester of pregnancy presented to the emergency department with fever, shortness of breath and muscle weakness. Initial investigations excluded pulmonary infection, thromboembolism, and cardiac dysfunction as the underlying cause of her symptoms. She developed deterioration in her level of consciousness due to carbon dioxide narcosis requiring invasive mechanical ventilation. Further workup revealed markedly elevated serum creatine kinase, abnormal electromyography and edema of her thigh muscles on magnetic resonance imaging. Diagnosis of polymyositis was confirmed by muscle biopsy. After receiving pulse steroid, intravenous immunoglobulins, and maintenance immunosuppressive therapy, our patient's respiratory muscle function improved and she was weaned off mechanical ventilation. Despite good maternal recovery from critical illness, the fetus developed intrauterine growth retardation and distress necessitating emergency cesarian section. CONCLUSIONS: New-onset polymyositis during pregnancy presenting with respiratory failure is rare. Early diagnosis and prompt initiation of therapy is necessary to improve fetal and maternal outcomes.
