• Journal of Electrodiagnosis and Neuromuscular Diseases
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• v.20 no.2
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• pp.148-152
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• 2018

Myotonic dystrophy type 1 (DM1) is an autosomal dominant multisystem disorder and one of the most common muscular dystrophies affecting adults. Charcot-Marie-Tooth (CMT) disease, a common hereditary neuropathy, is characterized by atrophy of the distal limbs and peripheral nerve abnormalities. The authors report a rare case involving a 24-year-old female who was diagnosed simultaneously with both DM1 and CMT1A based on the results of a nerve conduction study (NCS). The patient, who had previously been diagnosed with DM1, was admitted for lower extremity pain. Her electrodiagnostic examination continued to reveal severe sensorimotor demyelinating polyneuropathy, and a genetic study was performed to confirm whether she had other hereditary neuropathies, except DM1, that suggested CMT1A, the most common phenotype of CMT. Severe abnormalities in an NCS in a DM1 patient may suggest the incidental coexistence of hereditary neuropathies, and further evaluations, such as genetic studies, should be performed for proper diagnosis.

• Archives of Plastic Surgery
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• v.49 no.5
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• pp.608-610
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• 2022

In this article, we reported a single case of ischemic fasciitis in a young woman with a progressive immobilization due to a multifocal demyelinating disease of central nervous system, which appeared on an extensive pressure ulcer of the sacral region treated with 10 days of negative-pressure wound therapy (NPWT). Wound examination revealed a significant nontender brown neoformation (9 cm in length × 10 cm in width × 7 cm in height), fixed to the sacrum, presenting hard consistency, and grown in the central portion of the sacral pressure sore. The histologic examination showed central fibrinoid necrosis, and vascular and atypical fibroblastic proliferations, and a diagnosis of ischemic fasciitis was made. Ischemic fasciitis is a rare benign proliferation of atypical fibroblasts that occurs in physically weak patients with reduced mobility. In the literature, the relationship between the use of NPWT on pressure ulcers and the development of ischemic fasciitis is, to the best of our knowledge, not described yet.

• Journal of the Korean Society of Radiology
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• v.84 no.4
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• pp.964-969
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• 2023

Guillain-Barré syndrome (GBS) is an immune-mediated demyelinating polyneuropathy characterized by progressive, ascending, and symmetrical paralysis. It is known to be triggered by an antecedent infection or vaccination. Recently, GBS development following coronavirus disease 2019 (COVID-19) vaccination has been reported. Cranial neuropathies in typical GBS patients usually involve the facial and the lower cranial nerves (from IX to XII). We report a rare case of multiple cranial neuropathies involving trigeminal, abducens, and facial nerves in a patient who developed GBS following COVID-19 vaccination on the basis of obvious MRI features.

• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.580-584
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• 2007

Multiple sclerosis (MS) is a demyelinating disorder that affects discrete areas of the CNS, including the optic nerves, in a quite variable relapsing-remitting fashion over a prolonged period of time. Although MS is usually considered to be a disease that affects peoples in early to middle adulthood, children do develop multiple sclerosis. The frequency of MS onset before the age of 15 years is 2.7-5% of all cases, while MS onset during infancy and early childhood was observed to be 0.2-0.7% of all cases. We report here on a Korean case of a relapsing-remitting MS female child who was treated with four rounds of intravenous methylpredinsolone pulse therapy and preventive Interferon-$\beta$-1b ($Betaferon^{(R)}$).

• Annals of Clinical Neurophysiology
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• v.2 no.2
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• pp.81-88
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• 2000

Background : Guillain-$Barr{\acute{e}}$ syndrome(GBS) is characterized clinically by acute flaccid paralysis, areflexia, and albumino-cytologic dissociation. Based on electrophysiology and pathology, GBS can be divided into either predominantly demyelinating or predominantly axonal patterns. Objectives : The clinical and laboratory status of probable acute axonal GBS occurring at a mental hospital was evaluated. Methods : Eight schizophrenia patients with probable acute axonal GBS were analyzed. Results : The mean age of the patients was 38 years old. Most of the patients were men. All patients showed an acute ascending paraparesis and/or quadriparesis with areflexia, and all have a history of schizophrenia for 3~20 years. The diseases occurred predominantly in the summer and electrodiagnostic studies revealed axonal patterns. The patients were treated by supportive care, except one patient with intravenous immunoglobulin. The prognosis was improved in 3 ; no change in 4 and 1 became aggravated. One patient with acute motor-sensory axonal neuropathy had a recurrence after 10 months of the first attack. Conclusions : Axonal GBS has been considered uncommon clinically or electrophysiologically, but 8 probable acute axonal GBSs occurring at a mental hospital have been diagnosed in 3.5 years.

• The Journal of the Korea Contents Association
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• v.10 no.10
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• pp.237-245
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• 2010

Nerve conduction studies (NCS) are the most objective measure of nerve function and essential for the diagnosis of sub-clinical neuropathy in diabetes mellitus and diabetic polyneuropathy (DPN). This study evaluates the characteristic of electrophysiological abnormalities in DPN. Electrodiagnostic data from 120 patients with diabetic polyneuropathies and 77 control subjects were reviewed. Motor nerve conduction velocities (MNCV), distal motor latencies (DML), compound muscle action potential (CMAP) amplitudes, No potential frequency and conduction block were analyzed. Data were normalized based on normative reference values, and the proportion of nerves with abnormal values in the lower and upper limbs were evaluated. DPN was systemic demyelinating peripheral polyneuropathy and more severe abnormal nerve conduction was found in lower limbs than in upper limbs. The abnormal degree was more severe in peroneal nerve. It was no statistically significant difference of conduction block in control and DPN group. Our findings suggest that DPN had more common and severe peroneal nerve involvement in the motor nerve conduction studies (MNCS). These findings have important implications for the electrophysiological evaluation of DPN.

• Korean Journal of Biological Psychiatry
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• v.24 no.1
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• pp.1-9
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• 2017

The proton magnetic resonance spectroscopy ($^1H-MRS$) is a tool used to detect concentrations of brain metabolites such as N-acetyl aspartate, choline, creatine, glutamate, and gamma-amino butyric acid (GABA). It has been widely used because it does not require additional devices other than the conventional magnetic resonance scanner and coils. Demyelination, or the neuronal damage due to loss of myelin sheath, is one of the common pathologic processes in many diseases including multiple sclerosis, leukodystrophy, encephalomyelitis, and other forms of autoimmune diseases. Rodent models mimicking human demyelinating diseases have been induced by using virus (e.g., Theiler's murine encephalomyelitis virus) or toxins (e.g., cuprizon or lysophosphatidyl choline). This review is an overview of the MRS findings on brain metabolites in demyelination with a specific focus on rodent models.

• Investigative Magnetic Resonance Imaging
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• v.19 no.3
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• pp.186-190
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• 2015

Acute disseminated encephalomyelitis (ADEM) is a demyelinating and inflammatory condition of the central nervous system, occurring predominantly in white matter. ADEM involving the rhombencephalon without affecting the white matter is very rare. Here, we present an unusual case of ADEM involving only the rhombencephalon in a 4-year-old Asian girl. The patient complained of pain in the right lower extremities, general weakness, ataxia, and dysarthria. The initial brain CT showed subtle ill-defined low-density lesions in the pons and medulla. On brain MRI, T2 high signal intensity (T2-HSI) lesions with mild swelling were present in the pons, both middle cerebellar peduncles, and the anterior medulla. The initial diagnosis was viral encephalitis involving the rhombencephalon. Curiously, a cerebrospinal fluid (CSF) study revealed no cellularity, and negative viral marker findings. Three weeks later, follow up brain MRI showed that the extent of the T2-HSI lesions in the brain stem had decreased. After reinvestigation, it was found that she had a prior history of upper respiratory infection. In this case, we report the very rare case of a patient showing isolated involvement of the rhombencephalon in ADEM, mimicking viral rhombencephalitis on CT and MR imaging. ADEM can involve unusual sites such as the rhombencephalon in isolation, without involvement of the white matter or deep gray matter and, therefore, should be considered even when it appears in unusual anatomical areas. Thorough history taking is important for making a correct diagnosis.

• Clinical and Experimental Pediatrics
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• v.59 no.6
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• pp.271-275
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• 2016

Purpose: A limited number of studies have examined the link between F-wave abnormalities and clinical presentation in pediatric Guillain-$Barr{\acute{e}}$ syndrome (GBS). Therefore, this study examined the importance of F-wave abnormalities as a prognostic factor in pediatric GBS patients. Methods: The records and electrodiagnostic studies (EDS) of 70 GBS patients were retrospectively evaluated, and divided into 2 groups according to the results of EDS. Group A (n=33) presented with F-wave abnormalities, and group B (n=26) exhibited normal findings. We compared laboratory reports, clinical features, response to treatment, and prognosis between the 2 groups. Results: Motor weakness was the most frequently observed symptom for either group. Clinically, the incidence of fever and upper respiratory symptoms differed between the 2 groups, while the prevalence of abnormal deep tendon reflex (DTR) was significantly higher in group A than B (P<0.05). Patients diagnosed with GBS had received intravenous immunoglobulin treatment: 94% in group A and 58% in group B. Furthermore, significantly greater numbers of patients in group A showed H-reflex abnormalities and poor prognosis compared with group B (P<0.05). Conclusion: This study demonstrated that F-waves are a clinically important prognostic factor in GBS. F-wave abnormalities were associated with abnormal DTR and poor prognosis in patients. Limited studies have examined the link between F-wave abnormalities and clinical results; therefore, further randomized controlled studies are needed to confirm the clinical characteristics and efficacy of treatments.

• The Journal of Internal Korean Medicine
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• v.25 no.3
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• pp.609-614
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• 2004

Multiple Sclerosis(MS) is an acquired, demyelinating disease of the central nervous system. Clinically, it is characterized by episodes of focal disorder of the optic nerves, spinal cord, and brain, which remit to varying extent and recur over a period of many years. The average age at diagnosis is 30, typically starting between the ages of 15 and 50. Women are affected at least twice as often as men. It is more common in persons of northern European heritage and those living furthest from the equator. The diagnosis of MS is based on a history of multiple attacks of neurologic lesions over time that affect different parts of the central nervous system. A case of MS was confronted. The patient was treated with Cheongsimyonjaum-gami(淸心蓮字飮加味), YangMyung channel(陽明) and had significant improvement was seen.