• Clinical and Experimental Pediatrics
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• v.59 no.6
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• pp.276-279
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• 2016

Sjogren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder with worldwide incidence of 0.4 per 100,000 people. It is characterized by the triad of congenital ichthyosis, spastic diplegia or quadriplegia, and mental retardation. Herein we report a 2-year-old male child with SLS, asthma, and recurrent pneumonia. SLS was confirmed by a molecular genetics study that revealed a deletion mutation in the ALDH3A2 gene. An ALDH3A2 gene mutation results in dysfunction of the microsomal enzyme fatty aldehyde dehydrogenase and impaired metabolism and accumulation of leukotriene B4, which is a key molecule and a pro-inflammatory mediator in developing allergic diseases, especially asthma. An increased level of leukotriene B4 has been reported in SLS patients. As far as we are aware, this is the first report of SLS associated with asthma and recurrent pneumonia. In conclusion, pediatricians should be aware of and evaluate patients with SLS for possible associated asthma and allergic disorders.

• Journal of the Korean Society of Physical Medicine
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• v.6 no.2
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• pp.189-197
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• 2011

Purpose: Trunk muscle weakness in the children with cerebral palsy can lead to postural and alignment problems, breathing difficulties, and so on. Therefore, children with cerebral palsy can benefit from exercises that strengthen the muscles in their trunks. The purpose of this study was to investigate the effects of trunk muscle strengthening exercise on functional gross movement and balance ability in children with spastic diplegic cerebral palsy. Methods: We used single group repeated measure design in 8 children(four males, four females; aged 6~12 years; mean 8.3 years) with diplegia. The functional gross motor outcome measured by using the GMFM and balance ability of all children was measured by pediatric balance scale. All participants were alternately received trunk strengthening exercise and neurodevelopment treatment for 40 minutes twice per week during 8 weeks. Results: Significant and clinical meaningful improvement in functional gross motor and balance ability were shown. Conclusion: The results indicate that trunk strengthening exercise has a positive effect on both functional gross motor and balance ability in children with spastic diaplegic cerebral palsy.

• The Journal of Korean Physical Therapy
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• v.16 no.4
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• pp.115-128
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• 2004

The purpose of this study was to evaluate respiratory functions in relation to the gross motor functions(total value of GMFM), the difference of chest girth, and the changing position in spastic children. The respiratory functions(FVC, FEV1, $FEV1\%$, and PEF) were measured in the supine, the $45^{\circ}$semi-sitting, and the $45^{\circ}$sitting in 9 subjects. In the supine position, the mean difference of chest girth was $1.56{\pm}0.80cm$, the total value of GMFM was $45.41{\pm}17.79\%$. In the supine position, there was significant positive relationship in FVC-FEV1, FVC-PEF, and FEV1-PEF, but there was no significant relationship in GMFM and all respiratory functions. In the $45^{\circ}$semi-sitting, there was significant positive relationship in GMFM-FVC, FVC-FEV1, FVC-PEF, FEV1-PEF, and $FEV1\%-PEF$. In the $90^{\circ}$sitting, there was significant positive relationship in GMFM-FEV1, $GMFM-FEV1\%$, FVC-FEV1, FVC-PEF, and FEV1-PEF. In results of measured respiratory functions according to the postures, the supine position had highest value in all respiratory functions, but there were no significant (p<0.05).

• The Journal of Korean Physical Therapy
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• v.25 no.3
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• pp.155-159
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• 2013

Purpose: Circumference of the chest and waist can be one of clinical indicator to reflect respiratory function in children with cerebral palsy. In this study, we compared to differences in the chest/waist circumference and maximal phonation time between children with spastic diplegia and hemiplegia. Methods: Seventeen children with spastic diplegic and hemiplegic cerebral palsy were recruited, who were matched to gender, age, height, weight, and body mass index for control of the known factors affected to respiratory function. The chest/waist circumference and were measured in each group, when children took a breath at rest and at maximal voluntary inspiration/expiration. Results: No significant differences were found in the chest and waist circumference and expansion between the two groups. However, only in the waist expansion, children with diplegic CP were significantly lower extensibility of lung, compared to the other group. In comparison of the maximal phonation time, a significant lower score was shown in children with spastic diplegic CP, compared to children with hemiplegic CP. Conclusion: Our results indicated that children with spastic diplegic CP had smaller chest wall and waist, compared to children with spastic hemiplegic CP. In addition, they showed a shorter time for sustaining phonation than spastic hemiplegic CP did. Therefore, spastic diplegic CP will be required for careful monitor regarding respiratory function in rehabilitation settings.

• Journal of The Korean Society of Clinical Toxicology
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• v.13 no.1
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• pp.46-49
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• 2015

Carbon monoxide (CO) intoxication is a leading cause of severe neuropsychological impairments. Peripheral nerve injury has rarely been reported. Following are brief statements describing the motor peripheral neuropathy involved bilateral lower extremities of a patient who recovered following acute carbon monoxide poisoning. After inhalation of smoke from a fire, a 60-year-old woman experienced bilateral leg weakness without edema or injury. Neurological examination showed diplegia and deep tendon areflexia in lower limbs. There was no sensory deficit in lower extremities, and no cognitive disturbances were detected. Creatine kinase was normal. Electroneuromyogram patterns were compatible with the diagnosis of bilateral axonal injury. Clinical course after normobaric oxygen and rehabilitation therapy was marked by complete recovery of neurological disorders. Peripheral neuropathy is an unusual complication of CO intoxication. Motor peripheral neuropathy involvement of bilateral lower extremities is exceptional. Various mechanisms have been implicated, including nerve compression secondary to rhabdomyolysis, nerve ischemia due to hypoxia, and direct nerve toxicity of carbon monoxide. Prognosis is commonly excellent without sequelae. Emergency physicians should understand the possible-neurologic presentations of CO intoxication and make a proper decision regarding treatment.

• Journal of the Korean Academy of Clinical Electrophysiology
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• v.2 no.1
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• pp.9-17
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• 2004

The purpose of this study are to determine the proper radiographic measurements for hip deformity in spastic cerebral palsy patients, and the correlation of hip deformity with neurological involvement, ability of ambulation. Thirty children with cerebral palsy(22 males, 8 females) were evaluated by measurement of the migration index, acetabular index, center edge angle from bilateral hip APs(anterior posterior view). The result are as follows; 1. The incidence of hip dislocation, among the thirty children fifteen children were found to be dislocated, and more significantly high in non-walking group than in walking group(p<0.05) and in quadriplegia than in diplegia(p<0.05). 2. When compared to normal hip and dislocation hip, the migration index was significantly higher(p<0.01) and the center edge angle was lower in the dislocation hip than in the normal hip. 3. Correlation of radiological findings in right and left hip, the migration index and the center edge angle were highly correlated(p<0.01). We recommand regular intervals X-ray study for early diagnosis and management of hip dislocation in spastic cerebral palsy.

• Investigative Magnetic Resonance Imaging
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• v.23 no.2
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• pp.167-171
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• 2019

$M\ddot{o}bius$ syndrome is a rare congenital condition, characterized by abducens and facial nerve palsy, resulting in limitation of lateral gaze movement and facial diplegia. However, to our knowledge, there have been few studies on evaluation of cranial nerves, on MR imaging in $M\ddot{o}bius$ syndrome. Herein, we describe a rare case of $M\ddot{o}bius$ syndrome representing limitation of lateral gaze, and weakness of facial expression, since the neonatal period. In this case, high-resolution MR imaging played a key role in diagnosing $M\ddot{o}bius$ syndrome, by direct visualization of corresponding cranial nerves abnormalities.

• Annals of Clinical Neurophysiology
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• v.13 no.1
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• pp.44-47
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• 2011

Background: Miller-Fisher syndrome (MFS) is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered a variant form of Guillain-Barre syndrome. Although some cases of delayed-onset facial palsy in MFS have been reported, the characteristics of this facial palsy are poorly described in the literature. Methods: Between 2007 and 2010, six patients with MFS were seen at our hospital. Delayed facial palsy, defined as a facial palsy that developed while the other symptoms of MFS began to improve following intravenous immunoglobulin treatment, was confirmed in four patients. The clinical and electrophysiological characteristics of delayed facial palsy in MFS, as observed in these patients, are described here. Results: Four patients with delayed-onset facial palsy were included. Delayed facial palsy developed 8-16 days after initial symptom onset (5-9 days after treatment). Unilateral facial palsy occurred in three patients and asymmetric facial diplegia in one patient. The House-Brackmann score of facial palsy was grade III in one patient, IV in two patients, and V in one patient. None of the patients complained of posterior auricular pain. Facial nerve conduction studies revealed normal amplitude in all four patients. The blink reflex showed abnormal prolongation in two patients and the absence of action potential formation in two patients. Facial palsy resolved completely in all four patients within 3 months. Conclusions: Delayed facial palsy is a frequent symptom in MFS and resolves completely without additional treatment. Thus, standard treatment and patient reassurance are sufficient in most cases.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.29 no.4
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• pp.218-231
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• 2016

Objectives : To report the patient bilateral facial palsy, because it is unusual. Methods : One patient with bilateral facial palsy was selected. Herbal medicine(Cheunggansoyosan, bubidongyeom-2) was administered and acupuncture was applied for twice a day in 73 days. Facial function was evaluated by House-Brackmann scale, Yanagihara's scale and Standardization of muscular paralysis. Results : Cheunggansoyosan, bubidongyeom-2 and acupuncture treatment improved facial function after 73 days of treatment. Adverse effects were not reported. Conclusions : This study shows that the Korean medicine treatment was effective in improving bilateral facial palsy.

• Journal of Acupuncture Research
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• v.19 no.2
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• pp.238-249
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• 2002

Facial palsy is commonly encountered disease in the clinic but bilateral facial palsy is known as rare disease. Type of facial nerve paralysis include unilateral, recurrent ipsilateral, recurrent alternating and bilateral simultaneous palsies. Among the types, the reported incidence of bilateral simultaneous palsy is 0.3~2% of facial paralysis patients. We experienced I case of patient with bilateral simultaneous facial palsy that was concluded as bilateral bell's palsy. Objective : The purpose of this paper is to report the patient with bilateral facial palsy, who improved by oriental medical treatment. Another purpose is to review the current literature and to differential diagnosis of bilateral facial paralysis. Methods and Results : The patient was treated by acupuncture, herb medication and self-massage on facial muscle for 14 weeks. House-brackmann grading score was improved into I/I (Rt/Lt) from IV/IV. Conclusion : Through reviewing some literatures and reports, It is concluded that bilateral facial palsy was related to many other disorders and more ominous than unilateral facial palsy. therefore, its work-up should include a complete neurologic assesment and thorough evaluation. also, we consider that bilateral Bell's palsy can improve by oriental medical treatments.