• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.84-87
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• 2006

The MELAS (Mitochondrial Encephalomyopathy with Lactic Acidosis, and Stroke-like episodes) syndrome is one of the inherited mitochondrial disorder. We have experienced a 16-year-old girl with headaches and left hemianopsia. Diagnosis of MELAS syndrome with multiple brain parenchymal lesions was confirmed by gene study. The stroke-like lesion of MELAS syndrome showed significant improvement in radiological follow up study. Therefore, MRI findings in MELAS could be interpreted as metabolic cellular dysfunction rather than ischemic vasculopathy.

• Journal of Genetic Medicine
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• v.21 no.1
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• pp.36-40
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• 2024

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome is a maternally inherited mitochondrial disorder that usually affects the cerebral cortex and prevents high-energy demands from being met. Herein, we present the case of a male patient who rapidly developed multiple seizures, headaches, and altered mentality accompanied by severe metabolic acidosis and lactic acidosis. Initially, a brain imaging study confirmed stroke-like lesions (SLLs) only in the cerebellum. During follow-up, newly developed SLLs with lactic acidosis were observed in the basal ganglia (BG), cerebellum, and occipital lobe. The m.3243A>G variant had been found in the patient and MELAS was diagnosed, despite the BG and cerebellum being atypical locations for SLLs in MELAS. Since most cases of m.3243A>G variant MELAS show SLLs in the cerebral cortex, this case is unusual considering the location of the lesion. We emphasize that in the case of lactic acidosis accompanied by neurological symptoms, such as seizures, as in this case, MELAS should be included in the differential diagnosis, even if SLLs are observed in areas other than the cerebral cortex.

• The Journal of Internal Korean Medicine
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• v.43 no.2
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• pp.191-200
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• 2022

Intractable hiccups are those which persist for more than one month and can often occur in patients with disorders of the central nervous system, such as stroke, epilepsy, or brain tumor. An 80-year-old male patient undergoing conservative hemodialysis for diabetic nephropathy, recently developed paralysis and dysphagia due to central nervous system complications including stroke and epilepsy. He was admitted to the ◯◯ Oriental Medicine Hospital, and treated with herbal medicine (Gyulpijugyeo-tang, Jeonssiigong-san, and Ijin-tang) and electrical acupuncture for 30 days. No side effects were observed during hospitalization. The frequency and duration of hiccups were measured daily and found to reduce, and the effect persisted during his admission. The patient took no other nervous system drugs. In conclusion, Korean medicine like gyulpijugyeo-tang and acupuncture can be effective for patients with intractable hiccups who cannot use alternative drugs or treatment, because of other complications.

• Journal of Electrodiagnosis and Neuromuscular Diseases
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• v.20 no.2
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• pp.106-111
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• 2018

Transcranial magnetic stimulation (TMS) has been a gold standard for investigating central motor pathways in humans. Diffusion tensor imaging with fiber tractography (DTI FT) is known for its usefulness in detecting white matter lesion in vivo. We investigated the clinical usefulness of elucidating the integrity and continuity of corticospinal tract (CST) by combined use of TMS and DTI FT in this study. We report two cases who have presented with left hemiparesis and evaluated by both TMS and DTI FT; 10-year-old boy with Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like episode syndrome and 20-year-old woman with traumatic brain injury. Combined use of TMS and DTI FT successfully led to localize the brain lesion that might cause motor impairment in patients with abnormal signal intensities in MRI. The results of this study suggest that TMS and DTI FT might provide the detailed information between function and anatomy of the CST, complementarily.

• Archives of Plastic Surgery
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• v.34 no.4
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• pp.531-534
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• 2007

Purpose: Plastic surgeons are responsible for the management of spinal cord injury patients with upper and lower extremity reconstruction, pressure sore, and wounds. Derailment of autonomic nervous systems caused by injury to the spinal cord may result in fatal autonomic dysreflexia. Autonomic dysreflexia is a syndrome of massive imbalance of reflex sympathetic discharge occurring in patients with spinal cord lesion above the splanchnic outflow(T6). It is characterized by a sudden onset and severe increase in blood pressure and is potentially life threatening. The other classic symptoms are headache, chest pain, sweating, and bradycardia. In order to lower the blood pressure, it is important to remove the noxious stimulus for autonomic dysreflexia. If such symptoms last for more than 15 minutes despite conservative interventions, antihypertension drugs are recommended. Methods: In this case study, we report an autonomic dysreflexia case that developed in a 45 year-old tetraplegia patient with sacral pressure sore. When he got bladder irrigation, his blood pressure went up very high and his mentality became stuporous. He was sent to ICU for his blood pressure and mental care. ICU care made his vital sign stabilized and his mentality alert. Results: After the patient underwent proper treatment like inotropic agent, he was transferred to the general ward and his pressure sore on sacral area was coveraged with gluteus maximus myocutaneous advancement flap. Conclusion: If treatment is not effective, the patients have to undergo sudden, severe hypertension, which can cause stroke or death. To provide safe and effective care, plastic surgeons should be able to recognize and treat autonomic dysreflexia.

• IMMUNE NETWORK
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• v.6 no.1
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• pp.33-42
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• 2006

Background: Calcineurin plays a crucial role in T cell activation, cell growth, apoptosis, and angiogenesis, and its over-expression has been implicated in the pathogenesis of cardiomyopathy and stroke. However, the expression and function of calcineurin in the pathologic lesion of chronic inflammatory diseases, like rheumatoid synovium, remain to be defined. This study was aimed to determine the role of calcineurin in inflammatory arthritis and investigate the expression and function of calcineurin in the rheumatoid synovium and synoviocytes, the actual site of chronic inflammation. Methods: Immuno-histochemical staining using specific antibody to calcineurin was perfomed in the synovium of rheumatoid arthritis (RA). Fibroblast-like synoviocytes (FLS) from RA and osteoarthritis (OA) patients were isolated from RA and OA patients, and cultured with IL-1${\beta}$ and TNF-${\alpha}$ in the presence or absence of cyclosporin A, a calcineurin inhibitor. The calcineurin expression was assessed by phosphatase assay and Western blotting analysis. IL-6, -10, -17, matrix metalloproteinase (MMP)-1, -2, -3, and -9 released into the culture supernatants were measured by ELISA. After transfection with GFP-Cabin 1 gene into synoviocytes, the levels of IL-6 and MMPs were measured by ELISA. Results: Calcineurin was highly expressed in the lining layer of synovium and cultured synoviocytes of RA patients. The elevated calcineurin activity in the rheumatoid synoviocytes was triggered by proin flammatory cytokines such as IL-1${\beta}$ and TNF-${\alpha}$. In contrast, IL-10, an anti-inflammatory cytokine, failed to increase the calcineurin activity. The targeted inhibition of calcineurin by the over-expression of Cabin 1, a natural calcineurin antagonist, inhibited the production of IL-6 and MMP-2 by rheumatoid synoviocytes in a similar manner to the calcineurin inhibitor, cyclosporin A. Conclusion: These data suggest that abnormal activation of calcineurin in the synoviocytes may contribute to the pathogenesis of chronic arthritis, and thus provide a potential target for controlling inflammatory arthritis.