• Annals of Clinical Neurophysiology
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• 제19권1호
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• pp.68-70
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• 2017

Flail arm syndrome (FAS), known as one of the atypical amyotrophic lateral sclerosis (ALS) variants, has a similar clinical course and pathologic findings as ALS. Therefore it is difficult to differentiate between ALS and FAS at a glance. There are few reports involving individual analysis of FAS patients to date. The findings of polysomnography (PSG) in patient with FAS are not well known. We report a male FAS patient with review of literatures and several issues related to the diagnostic process.

• 근관절건강학회지
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• 제13권2호
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• pp.130-139
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• 2006

Purpose: This study was designed to present preliminary data for the development of appropriate nursing care system for the patients with ALS by analyzing their physical function. Method: The clinical data of 36 ALS patients, who visited ALS Clinic of H University Hospital in Seoul, were collected from January, 2006 to August, 2006. To determine the physical function, Norris ALS scale and Appel ALS Rating Scale were used. The data were analyzed by frequency, percentage, mean, standard deviation, range, t-test, ANOVA, using SPSS PC program. Results: The mean score of physical activity, muscle strength, upper extremity function, lower extremity function was 18.08, 27.72, 25.94, 25.19 respectively. There were significant differences in physical activity, muscle strength, and upper extremity function according to sender and comorbid disease(diabetes). Although sites of symptom onset were not statistically significant with all physical function, patients with bulbar onset showed relatively severe physical disabilities. Conclusion: The preliminary data on physical function of patients with ALS would be helpful for the development of ALS nursing guideline system.

• Annals of Clinical Neurophysiology
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• 제10권1호
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• pp.62-65
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• 2008

We report a patient with amyotrophic lateral sclerosis (ALS) who developed a pneumothorax after needle electromyography (EMG), probably of the intercostal muscles. Needle EMG on intercostal muscles has a high risk of pneumothorax, not only because electromyographers are unfamiliar to its technique, but also due to its close anatomical location to the pleural cavity. In our patient, advanced intercostal muscle atrophy due to disease process would have increased the risk further.

• Annals of Clinical Neurophysiology
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• 제6권2호
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• pp.85-91
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• 2004

Background: Needle electromyography (EMG) and motor evoked potential (MEP) of the genioglossus (tongue) are difficult to perform in evaluations of the craniobulbar region in amyotrophic lateral sclerosis (ALS). Therefore, we investigated the yields of needle EMG and MEP recorded from the upper trapezius, since it receives innervation from the lower medulla and upper cervical cord. Methods: Needle EMG and MEP of the upper trapezius were obtained in 17 consecutive ALS patients. The needle EMG parameters recorded included abnormal spontaneous activity and motor unit action potential (MUAP) morphology. An upper motor neuron (UMN) lesion was presumed when either response to cortical stimulation was absent, or the central conduction time was delayed (>mean+2SD). Results: Of the five patients with bulbar-onset ALS, four had abnormalities in the upper trapezius and four in the tongue by needle EMG. In contrast, of the 12 patients with limb-onset ALS, 11 had abnormalities in the upper trapezius, and only five in the tongue. When MEP was performed, it was found that three of the five patients with bulbar symptoms and three of the six patients with isolated limb involvement had abnormal MEP findings. Conclusions: Electrophysiological studies of the upper trapezius are more sensitive those of the tongue in patients without bulbar symptoms. Thus, needle EMG and MEP of the upper trapezius are alternative tools for assessing bulbar and rostral neuraxial involvement in the diagnosis of ALS.

• 대한약침학회지
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• 제13권1호
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• pp.121-128
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• 2010

Objectives : This study was to investigate the effects of Oriental medical treatment on ALS. Methods : We investigated 12 ALS patients which were admitted to Gwang-Ju O.M.hospital for 3 months and follow-up at 8 months after discharge. All patients were treated by SAAM-acupuncture, herb medication, Bee venom Pharmacopuncture therapy, Needle-embedding therapy, etc and after discharge self-therapy at home. We evaluated patients using the Amyotrophic lateral sclerosis Functional Rating Scale-Revised(ALSFRS-R), Medical Research Council (MRC) Scale. Results : After 1 month, mean ALSFRS-R score of patients was $29.08{\pm}7.99$, 2 months $28.70{\pm}7.17$, 3 months $28.16{\pm}8.23$, 1 year $21.33{\pm}9.93$ and mean MRC Scale of patients was $25.34{\pm}8.45$, 2 months $25.34{\pm}8.45$, 3months $21.56{\pm}9.20$. But in both cases, the variation was not statistically significant. Conclusions : We think that the results of this case be a pilot study that proves the effect of Oriental Medical treatment on ALS.

• 대한약침학회지
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• 제12권1호
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• pp.53-65
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• 2009

Objectives : This study was to investigate the effect of Oriental medical treatment on ALS. Methods : We investigated 12 ALS patients which were admitted to Gwang-Ju O.M. hospital from Oct. 14, 2008 to Nov. 14, 2008. All patients were treated by SAAM-acupuncture, herb medication, Bee venom Pharmacopuncture therapy, Needle-embedding therapy, etc. We evaluated patients using the Amyotrophic lateral sclerosis Functional Rating Scale-Revised(ALSFRS-R), Medical Research Council (MRC) Scale. Results : After 30 days, mean ALSFRS-R score of patients was improved from $28.42{\pm}7.83$ to $29.08{\pm}7.99$, and mean MRC Scale of patients was improved from $24.79{\pm}8.37$ to $25.34{\pm}8.45$. But in both cases, the variation was not statistically significant. After 30 days, mean ALSFRS-R score and mean MRC Scale of patients was more improved in subjects with bulbar-onset, onset age: 51-60yrs., disease duration: 24-48mo. And the results showed partially significant difference. Conclusions : We think that the results of this case be a pilot study that proves the effect of Oriental Medical treatment on ALS.

• Journal of Acupuncture Research
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• 제30권2호
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• pp.73-79
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• 2013

Objectives : Sialorrhea in amyotrophic lateral sclerosis patients is a cause of death due to aspiration pneumonia as well as reduces the patient's quality of life. We report the changes that appeared in the sialorrhea treated by using trihexyphenidyl and Korean medical treatments. Methods : We treated amyotrophic lateral sclerosis patient with sialorrhea by using trihexyphenidyl a known antiparkinsonian agent and Korean medical treatments such as acupuncture, pharmacopuncture and herbal medicine. The salivation rate was checked with visual analogue scale(VAS). Results : There was a more than 50 % decrease in salivation in this case. Owing to the constipation, trihexyphenidyl was stopped after which only Korean medical treatments were provided. Over which a lasting decrease in salivation could be seen. Conclusions : Existing treatments would cause several considerable side effects and have difficulty in being applied in domestic clinics. In this respect, we suspect that our findings could open up new clinical guideline possibilities. We should solve the limitations of this case study and conduct more studies.

• Journal of Acupuncture Research
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• 제24권4호
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• pp.225-235
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• 2007

2007년 2월 20일부터 3월 12일까지 대전대학교 둔산 한방병원에 입원한 근위축성 측삭 경화증 환자 1례에 대한 임상 고찰을 통하여 다음과 같은 결론을 얻었다. 1. 본 증례의 환자는 한의학적으로 위증으로 진단 하였으며 폐열엽초(肺熱葉焦), 기혈양허(氣血兩虛)로 변증하였다. 2. 독취양명(獨取陽明)치법을 기준으로 비위경(脾胃經)의 경혈 위주로 자침하였고 입원초기의 한약은 기혈대보(氣血大補)하는 십전대보탕(十全大補湯), 자신양혈건보탕(滋腎養血健步湯)을 사용하였으나 입원 중기부터는 객담, 인후부 불편감을 사지무력감보다 심하게 호소하여 우담리습(祐痰利濕)의 효능이 있는 보음전(補陰煎), 절제화담환(節齊化痰丸)을 처방하였다. 3. 환자는 입원 초기 객담이 줄어들면서 연하작용도 완화되었으나 입원후기에는 객담이 다시 증가하여 연하 및 호흡곤란을 호소하였다. 4. 근육의 위축 속도 및 정도를 파악하기 위하여 1주일에 1회 하지둘레를 확인하였으며 입원 첫 주보다 퇴원 주에 1cm 가량 감소하였다. 5. 우상지부 악력은 입원 초기 6kg이었으나 입원 후기에는 11kg까지 증가하였다. 6. ALS는 아직 뚜렷한 치료법이 없는 상태로 향후 적극적인 한방치료에 대한 연구가 필요할 것이라고 사료된다.

• 척추신경추나의학회지
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• 제5권2호
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• pp.103-111
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• 2010

ALS를 진단받았으며 2010.5.11부터 2010.6.10까지 ${\bigcirc}{\bigcirc}$한방병원에 입원한 요통을 호소하는 남환에 대하여 화침치료, 전침치료, 침치료, 구치료, 약물치료, 이학요법 등을 시행한 결과 K-ODI, VAS, K-ALSFRS-R 등의 평가 항목에 대하여 호전 경향을 보였다. ALS는 기본적으로 진행성 경과를 밟는 질환이기 때문에, 병의 진행을 늦추는 것과 대증치료를 통해 삶의 질을 높이는 것이 중심이 된다고 볼 수 있다. 본 환자의 경우 상기 한의학적 처치에 의해 일정한 효과가 나타났다. ALS에서 수반되는 요통의 호전에 의해 기능수행능력이 향상된 결과라고 볼 수 있다. 이는 한의학적 치료 방법이 ALS 환자의 치료에 있어서 통증을 감소시키고 일상생활에서의 기능수행능력을 향상시킴으로써 삶의 질을 높이는 대중치료의 역할을 수행할 수 있다는 단서라고 할 수 있다. 치료효과의 검증을 위해서는 추후 지속적인 증례 연구가 필요할 것으로 사료된다.

• The Korean Journal of Physiology and Pharmacology
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• 제3권1호
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• pp.101-117
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• 1999

Amyotrophic lateral sclerosis (ALS) is a degenerative neuromuscular disease of unknown etiology in which the upper and lower motor neurons are progressively destroyed. Recent evidences support the role of autoimmune mechanisms in the pathogenesis of ALS. This study investigated the effects of sera from ALS patients on neuromuscular transmission in phrenic nerve-hemidiaphragm preparations and on calcium currents of single isolated dorsal root ganglion (DRG) cells in mice. Mice were injected with either control sera from healthy adults or ALS sera from 18 patients with ALS of sporadic form, for three days. Miniature end plate potential (MEPP) and nerve-evoked end plate potential (EPP) were measured using intracellular recording technique and the quantal content was determined. Single isolated DRG cells were voltage-clamped with the whole-cell configuration and membrane currents were recorded. Sera from 14 of 18 ALS patients caused a significant increase in MEPP frequency in normal Ringer's solution $(4.62{\pm}0.14\;Hz)$ compared with the control $(2.18{\pm}0.15\;Hz).$ In a high $Mg^{2+}/low\;Ca^{2+}$ solution, sera from 13 of 18 ALS patients caused a significant increase in MEPP frequency, from $2.18{\pm}0.31$ Hz to $6.09{\pm}0.38$ Hz. Sera from 11 of 18 patients produced a significant increase of nerve-evoked EPP amplitude, from $0.92{\pm}0.05$ mV to $1.30{\pm}0.04$ mV, while the other seven ALS sera did not alter EPP amplitude. In the ALS group, EPP quantal content was also elevated by the sera of 14 patients (from $1.49{\pm}0.07$ to $2.35{\pm}0.07).$ MEPP frequency and amplitude in wobbler mouse were $4.03{\pm}0.53$ Hz and $1.37{\pm}0.18$ mV, respectively, which were significantly higher than those of wobbler controls (wobblers without the symptoms of wobbler). Sera from ALS patients significantly reduced HVA calcium currents of DRG cells to 42.7% at -10 mV. Furthermore, the inactivation curve shifted to more negative potentials with its half-inactivation potential changed by 6.98 mV. There were, however, significant changes neither in the reversal potential of $I_{Ca}$ nor in the I-V curve. From these results it was concluded that: 1) The serum factors of sporadic ALS patients increase neuromuscular transmission and can alter motor nerve terminal presynaptic function. This suggests that ALS serum factors may play an important role in the early stage of ALS, and 2) Calcium currents in DRG cells were reduced and rapidly inactivated by ALS sera, suggesting that in these cells, ALS serum factors may exert interaction with the calcium channel.