• Annals of Clinical Neurophysiology
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• v.19 no.1
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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.

• Journal of muscle and joint health
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• v.13 no.2
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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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• v.10 no.1
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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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• v.6 no.2
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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.

• Journal of Pharmacopuncture
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• v.13 no.1
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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.

• Journal of Pharmacopuncture
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• v.12 no.1
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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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• v.30 no.2
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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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• v.24 no.4
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• pp.225-235
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• 2007

Amyotrophic Lateral Sclerosis(ALS) is Motor Neuron Disease(MND) that reveals muscle relaxation, bulbar palsy, extremities weakness, Pneumonia, in severe case, leads to death. Objectives : Amyotrophic Lateral Sclerosis is one of the incurable disease. In Oriental medicine, Wei symptom is similar as Amyotrophic Lateral Sclerosis, so we diagnosed it as Wei symptom and treated in Oriental medical system. Methods : The patient was treated by acupunture, moxibustion, herb medication, physical treatment. The improvement of the patient was judged by Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS), Grasping power on right arm, circumference of thigh and calf. Result : The patient had better condition for a while but the sputum irritated breathing and the day before he discharged vital sign was not stable and could not breath well. Conclusion : It is necessary to have more examination about the incurable syndromes such as Amyotrophic Lateral Sclerosis, and keep the patient's life better and expand their lives.

• The Journal of Churna Manual Medicine for Spine and Nerves
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• v.5 no.2
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• pp.103-111
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• 2010

Objectives : This study was performed to report the effect of oriental medical treatment in the lower back pain(LBP) with amyotrophic lateral sclerosis(ALS). Methods : A 32-years-old man who diagnosed as ALS was admitted with LBP. We treated him by burning acupuncture, herbal medicine, acupuncture, electroacupuncture, moxibustion, cupping therapy and physical therapy from 11th May 2010 to 10th June 2010. Results : After treatment, there was improvement in LBP. Pain Intensity estimated by visual analog scale(VAS) as percentile Pain Intensity Difference(PID), Korean Owestry Disbility Index(KODI) was also improved. Korean version of Amyotrophic lateral sclerosis Functional rating scale-revised(K-ALSFRS-R) shows that the patient's physical ability has been improved. Conclusions : Our study suggests that oriental medical treatment are significantly effective in the LBP due to ALS. And further studies will be aid to identify underlying mechanism of treatment.

• The Korean Journal of Physiology and Pharmacology
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• v.3 no.1
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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.