Subject(s)
COVID-19 , Leigh Disease , Humans , COVID-19/complications , Vasoconstriction , SARS-CoV-2 , Syndrome , VaccinationABSTRACT
A 26-year-old woman developed a sudden headache, ptosis, and diplopia. Magnetic resonance imaging and angiography demonstrated a symmetrical lesion from the midbrain to the brainstem, involving the solitary nucleus and multifocal cerebral artery narrowing. Reversible cerebral vasoconstriction syndrome (RCVS) was suspected, and the patient improved after vasodilatation. Leigh syndrome was suspected due to the elevated serum pyruvate level, so mitochondrial DNA was analyzed, and an m.9176T>C mutation was detected. The final diagnosis was adult-onset Leigh syndrome manifesting as RCVS. An uncontrolled baroreflex due to a solitary nuclear lesion or endothelial dysfunction may have contributed to her unique presentation.
Subject(s)
Cerebrovascular Disorders , Headache Disorders, Primary , Leigh Disease , Vasospasm, Intracranial , Female , Humans , Adult , Magnetic Resonance Angiography/methods , Leigh Disease/diagnosis , Leigh Disease/genetics , Vasoconstriction , MutationABSTRACT
We report the case of a 73-year-old woman with progressive gait disturbance for several months and an impaired standing ability. Her symptoms were lower-limb-dominant spastic tetraplegia with normal muscle tonus, hyperreflexia with positive Babinski sign, hypothermesthesia and hypalgesia in bilateral lower limbs, and dysesthesia in the fingers of both hands. Magnetic resonance (MR) images revealed a long lesion extending to nearly the entire spinal cord, and chest computed tomography (CT) revealed diffuse multiple small nodular lesions in the lower parts of the right and left lungs. High titers of human T-cell lymphotropic virus (HTLV)-1 antibodies in the serum and CSF, large amount of HTLV-1 provirus and neopterin in the CSF, and lymphocytic infiltration around bronchioles in pulmonary biopsy specimens indicated the presence of HTLV-1-associated myelopathy (HAM) with HTLV-1-associated bronchiolo-alveolar disorder (HABA). Her neurologic symptoms and the lesions in the spinal cord and lungs rapidly improved after corticosteroid pulse therapy. Rapid progressive HAM should be considered as a differential diagnosis of subacute spastic paraparesis with long spinal cord lesions. Further, careful investigation to detect pulmonary lesions, which are reported to be asymptomatic in most cases, is useful to confirm a diagnosis of HAM.
