COVID-19-Associated Acute Multi-infarct Encephalopathy in an Asymptomatic CADASIL Patient

By Tianshu Zhang, Ellen Hirsh, Shadi Zandieh & Michael B. Rodricks
First Online: 06 October 2020

COVID-19 is a new disease caused by the SARS-CoV-2 virus. First described in Wuhan, China, the scope and speed with which the disease has spread has placed healthcare systems around the world under pressure. Afflicted patients typically present with fever, cough, myalgia, and eventually dyspnea. Despite the characteristic presentation of COVID-19, we have frequently noted atypical symptoms including gastrointestinal disturbances and neurological symptoms including headache, altered mental status, loss of olfaction, and seizures. We present the case of a patient who presented with significant neurological symptoms.

A right-handed female in her early 40s presented to the emergency department after she developed dysphagia, dysarthria, and encephalopathy 2 days prior to admission. She had a history of well-controlled hypertension and dyslipidemia. The patient had no past medical history of stroke, migraines, visual disturbances, or any neurological disease processes. She had no previous brain imaging studies. There was also no family history of stroke, dementia, migraines with aura, or other neurological disorders.

Eleven days prior to admission, the patient began suffering from a headache and myalgia. She was seen by her primary care physician and treated as an outpatient with a course of azithromycin. She had a negative influenza swab and a negative rapid strep test at the office. She was, however, not checked for COVID-19 due to the lack of available testing. The patient’s sister, with whom she resided, had recently returned from a trip abroad. The day after returning, the sister developed a headache and myalgias which were mild, lasted 4 days, and were self-limited. Her sister did not seek any medical care. The patient fell ill 4 days after her sister’s return with similar symptoms of a headache and myalgias. After developing lethargy, dysphagia, and speech difficulties, she was brought to the emergency department.

The physical examination revealed a patient in moderate acute distress. She was febrile with a temperature of 102.2°F. Her blood pressure was 140/80 with a heart rate of 106 and a respiratory rate of 27. The room air oxygen saturation was 92%. Neurologically, she was awake and alert and followed commands although sluggishly. She had difficulty with her speech with components of both dysarthria and expressive aphasia, difficulty handling her secretions, and dysphagia. There was no meningismus which could be elicited. Her pupils were equal round and reactive, but she showed a right gaze preference and a mild left facial droop. She had mildly decreased but equal bilateral strength. The deep tendon reflexes were preserved. The remainder of the examination was only notable for diffuse rhonchi on auscultation of her lungs.

Initial laboratory studies showed a mild leukocytosis with lymphopenia. The chest X-ray demonstrated patchy consolidation in the right lower lung. A non-contrast computed tomography (CT) of the head showed no evidence of intracranial hemorrhage, but there were multifocal patchy areas of white matter hypoattenuation (Fig. 1). A lumbar puncture was performed to clarify the diagnosis and to exclude central nervous system infection. Cerebrospinal fluid (CSF) analysis revealed normal cell counts, protein, and glucose. A polymerase chain reaction (PCR) panel for meningitis and encephalitis, including herpes simplex 1 and 2, human herpes 6, Cryptococcus, and Varicella Zoster virus, was entirely negative as were bacterial cultures. A Lyme titer was negative. An electroencephalogram (EEG) did not show electrical evidence of seizures. The COVID-19 PCR test of a nasal swab became available 2 days after admission and detected the novel coronavirus (SARS-CoV-2) target nucleic acid. The COVID-19 PCR test of CSF was negative (Cepheid GeneXpert System).

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