Abstract
BackgroundNeuromyelitis Optica (NMO) is an inflammatory demyelinating disease that mainly affects optic nerves and spinal cord. Besides, loss of motor and cognitive function has been reported as important symptoms of disease.ObjectiveHere we investigated the mitochondrial dysfunction and metabolic alterations in NMO patients and evaluate their correlation with disease progress, disability and cognitive impairment.MethodsThe individuals (12 controls and 12 NMO) were assessed for disease severity by expanded disease status scale (EDSS), cognitive function via symbol digit modalities test (SDMT) and fine motor disability by 9-hole peg test (9-HPT). We have measured Sirtuin 1 (SIRT1), SIRT3, mitochondrial complex I, complex IV, aconitase and α-ketoglutarate dehydrogenase (α-KGD) activity in peripheral blood mononuclear cells (PBMCs). Furthermore, SIRT1, pyruvate, lactate and cytochrome c (Cyt c) were determined in plasma.ResultsOur results exhibited increased 9-HPT time in NMO patients. 9-HPT results correlated with EDSS; and SDMT negatively correlated with disease duration and number of attacks in patients. Investigation of PBMCs of NMO patients exhibited a decrease of mitochondrial complex I and IV activity that was significant for complex IV. Besides, complex I activity was negatively correlated with 9-HPT time in NMO group. In the plasma samples, a correlation between pyruvate to lactate ratio and EDSS in NMO patients was found and a negative correlation between Cyt c concentration and SDMT was detected.ConclusionOur data support the hypothesis that mitochondrial dysfunction occurred in the CNS and the peripheral blood may contribute to disease progress, disability level and the cognitive impairment in NMO patients.
Highlights
Neuromyelitis optica (NMO) is known as an autoimmune disorder of the central nervous system (CNS) which attack optic nerve and spinal cord
Our results exhibited increased 9-hole peg test (9-HPT) time in NMO patients. 9-HPT results correlated with expanded disease status scale (EDSS); and Symbol Digit Modalities Test (SDMT) negatively correlated with disease duration and number of attacks in patients
Investigation of peripheral blood mononuclear cells (PBMCs) of NMO patients exhibited a decrease of mitochondrial complex I and IV activity that was significant for complex IV
Summary
Neuromyelitis optica (NMO) is known as an autoimmune disorder of the central nervous system (CNS) which attack optic nerve and spinal cord. The term NMO spectrum disorders (NMOSDs) was introduced to mention AQP4 IgG-seropositive patients [1]. The major lesions and tissue atrophy of NMO patients is in optic nerve and spinal cord, regional brain atrophy has been reported in this disease [4,5,6]; for instance, investigations demonstrated that NMO patients suffer cognitive dysfunction [6,7]; with main cognitive deficits in long-term memory, speed of information processing, attention and executive functions. Neuromyelitis Optica (NMO) is an inflammatory demyelinating disease that mainly affects optic nerves and spinal cord. Loss of motor and cognitive function has been reported as important symptoms of disease
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