• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.208-211
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• 2006

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.

• Journal of Genetic Medicine
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• v.18 no.1
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• pp.44-47
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• 2021

Cerebral creatine deficiency syndrome (CCDS) is a disorder where a defect is present in transport of creatine in the brain. Creatine plays an essential role in the energy metabolism of the brain. This is a genetic disorder, autosomal recessive or X linked, characterized by intellectual disability, speech and language delay, epilepsy, hypotonia, etc. Until recently very few number of cases have been reported. Here we report a case of 1.5-year-old boy who had epilepsy (epileptic spasm and generalized tonic clonic seizure), intellectual disability, microcephaly, hypotonia and speech delay. His magnetic resonance imaging of brain showed cortical atrophy and electroencephalography showed burst suppression pattern. The diagnosis was confirmed by clinical exome sequencing which showed novel mutation of SLC6A8+ in exon 9, suggestive of X linked recessive CCDS. The patient was then treated with glycine, L-arginine and creatine monohydrate with multiple antiepileptic drugs.

• Physical Therapy Korea
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• v.13 no.3
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• pp.57-66
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• 2006

Ischemic stroke results from a transient or permanent reduction in cerebral blood flow that is restricted to the territory of a major brain artery. Thus, this study was performed to examine (1) the effects of swimming exercise on the improvement of muscle atrophy, and (2) exercise and HSP 70 expression in an ischemic stroke model induced by middle cerebral artery occlusion. The results of this study were as follows: One week after ischemic stroke was induced, changes appeared in the muscle weight of the gastrocnemius muscle due to muscle atrophy in the affected side. Group II showed statistically significant difference from group III eight weeks after ischemic stroke was induced. (p<.05). One week and eight weeks after ischemic stroke was induced there was significant decrease in the relative muscle weight of the gastrocnemius muscle in each group except Group IV, while there was statistically significant increase in group II eight weeks after ischemic stroke was induced, compared to group III (p<.05). For neurologic exercise behavior tests, Group II generally had the highest score, compared to other groups. In immunohistochemical observations, Group II showed a decrease in HSP 70. The above results suggest that swimming exercise improved muscle atrophy, changed the HSP 70 expression of ischemic stroke in rats, and contributed to the improvement of exercise function.

• The Journal of Korean Physical Therapy
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• v.18 no.3
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• pp.9-21
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• 2006

Purpose: This study was intended to examine the effects of electroacupuncture and therapeutic exercise on muscle atrophy and exercise function in an ischemic stroke model induced by middle cerebral artery occlusion. Methods: This study selected 120 Sprangue-Dawley rats, 8-week of age, divided them into six groups, and assigned 5 rats to each group. Experiments were conducted for 1, 3 days, 1, and 8 weeks, respectively. Group I was a group of electroacupuncture and therapeutic exercise after inducing ischemic stroke; Group II was a group of therapeutic exercise after inducing ischemic stroke; Group III was a group of electroacupuncture after inducing ischemic stroke; Group IV was a sham group of electroacupuncture after inducing ischemic stroke; Group V was a control group and Group VI was a sham group without ischemic stroke. In each group, changes in weight of muscle and relative muscle of TA muscle, neurologic motor behavior test, histologic observations were observed and analyzed. Results: For the changes in muscle weight of unaffected and affected sides of TA muscle, muscle atrophy was seen in an affected side 3 days after ischemic stroke was induced. There was statistically significant difference in Group I 1 week and 8 weeks after ischemic stroke was induced, compared to Group V (p<0.05). For the changes in relative muscle weight of unaffected and affected sides of tibial anterior muscle, there was significant decrease in each group 3 days after ischemic stroke was induced, compared to Group IV, while there was statistically significant increase in Group I 1 week after ischemic stroke was induced, compared to Group V (p<0.05). For neuologic exercise behavior test, Group I generally had the highest score, compared to other groups. Conclusion: electroacupuncture and therapeutic exercise may improve muscle atrophy and change in histologic observations expression of ischemic stroke rats and contribute to the improvement of exercise function.

• The Korean Journal of Nuclear Medicine
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• v.22 no.2
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• pp.163-170
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• 1988

8 patients of delayed CO sequelae were evaluated using Brain CT and $^{99m}Tc-HMPAO$ SPECT. The results were as follows; 1) CT findings of delayed CO sequleae were bilateral low density lesion in globus pallidus (l pt.), diffuse low density in white matter with bilateral low density in white matter (l pt.), diffuse low density in white matter with bilateral low density in globus pallidus (l pt.), diffuse low density in white matter with cortical atrophy (l pt.), bilateral low density in globus pallidus and diffuse low density in white matter with cortical atrophy (l pt.) and normal in 3 pts. 2) $^{99m}Tc-HMPAO$ Brain SPECT findings of delayed CO sequelae were decreased regional cerebral blood flow (rCBF) in frontal (1 among 8 pts.), frontal and basal ganglia (3 among 8 pts.), and diffuse patch decreased rCBF pattern (4 among 8 pts.) 3) $^{99m}Tc-HMPAO$ Brain SPECT study was well correlated with neurologic symptoms and signs in delayed CO sequelae. Our results may suggest that reduced cerebral blood flow contributes to the development of delayed CO sequelae.

• The Korea Journal of Herbology
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• v.23 no.2
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• pp.159-168
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• 2008

Objectives : The present study has been undertaken to investigate the effects of Dipsaci Radix on Muscle Fiber Atrophy and MyoD Expression in Gastrocnemius of MCAO Rats Methods : In order to investigate effects of Dipsaci radix on the skeletal muscle atrophy following stroke, cerebral infarct was induced by the middle cerebral artery occlusion (MCAO) in the rats. Water extract of Dipsaci radix (184.4 mg/100 g) was treated for 4 weeks, once a day orally, after the MCAO. Effects were evaluated with muscle fiber type composition and cross-sectioned area of muscle fibers in gastrocnemius of the unaffected & affected hind limbs. And MyoD protein expression in gastrocnemius was demonstrated with immunohistochemistry and western blotting. Results : Obtained results were as follows; 1. Infarct volume was not attenuated by Dipsaci radix treatment in the MCAO rats. 2. At the affected-side hind limb of the MCAO rats, the increase of type-I fibers and the decrease of type-II fibers were induced by Dipsaci radix treatment. 3. At the affected-side hind limb of the MCAO rats, decreases of cross-sectioned areas of type-I and type-II fibers were attenuated by Dipsaci radix treatment. 4. At the affected-side hind limb of the MCAO rats, MyoD positive cells were increased by Dipsaci radix treatment. 5. At the affected-side hind limb of the MCAO rats, MyoD expressions were increased by Dipsaci radix treatment. Conclusions : These results suggest that Dipsaci radix has a protective effect against muscle atrophy, through the inhibition of the muscle cell apoptosis, following the central nervous system demage.

• Physical Therapy Korea
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• v.14 no.1
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• pp.1-10
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• 2007

This study was designed to examine the effects of electroacupuncture and treadmill exercise on the improvement of muscle atrophy and Brain-Derived Neurotrophic Factor (BDNF) expression in an ischemic stroke model induced by middle cerebral artery occlusion. This study selected 120 Sprangue-Dawley rats, divided them into six groups, and assigned 5 rats to each group. Experiments were conducted for 1, 3 days and 1, 8 weeks, respectively. In each group, changes in weight of muscle and relative muscle of tibialis anterior muscle, histologic observations, and BDNF expression were observed and analyzed. For the changes in muscle weight of unaffected and affected sides of tibialis anterior, muscle atrophy was expressed in an affected side 3 days after ischemic stroke was induced. There was a statistically significant difference in Group VI 1 and 8 weeks after ischemic stroke was induced, compared to Group II (p<.05). For the changes in relative muscle weight of unaffected and affected sides of tibial anterior muscle, there was significant decrease in each group 3 days after ischemic stroke was induced, compared to Group I, while there was a statistically significant increase in Group VI 1 week after ischemic stroke was induced, compared to Group II (p<.05). For neurologic exercise behavior test, Group VI generally had the highest score, compared to other groups. The results of the behavior test suggests that 8 weeks after ischemic stroke was induced, Group VI improved in degeneration and inflammation of muscle fiber and decreased in destruction of nerve cells and cerebral infarction, thus indicating a similar state of muscle fiber and brain tissue in Group I. In immunohistochemical observations, Group 1 week showed increase in BDNF. Based on these results, electroacupuncture and treadmill exercise may improve muscle atrophy and change in BDNF expression of ischemic stroke rats and contribute to the improvement of exercise function.

• Journal of Korean Neurosurgical Society
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• v.52 no.5
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• pp.498-500
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• 2012

Cranioplasty is performed using autograft and allograft materials on patients to whom craniectomy was applied previously due to the facts that, this region is open to trauma and the scalp makes irritation and pressure onto the brain paranchyma causing brain atrophy and convulsions. Dramatical improvement of neurological deficits, control of convulsions and partial prevention of cerebral atrophy are achieved after these operations. One of the most important complications of cranioplasty is late infection. Here, we report a 43-year-old male patient admitted with the history of purulant discharge from the right temporal incission site for one year to whom cranioplasty had been performed with allograft material 20 days after craniectomy which had been performed in 1989. Allograft cranioplasty material was removed and cranioplasty was performed using new allograft material with the diagnosis of late cranioplasty infection.

• Journal of Veterinary Clinics
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• v.17 no.2
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• pp.470-474
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• 2000

A Shih Tzu Puppy had clinical onset of anotexia crying and progression of neurological sings when enlargement of the cranial vault at 1 month old and died after showing clinical signs during 2 months period. Radiological and pathological examinations were performed. Radiological findings were homogeneous appearance of the calvaria with cortical thinning, loss of the normal convolutional skull markings and persistent fontanelles. Grossly enlargement of the cranial vault thinning of the bone and defective closure of the fontanelles were also observed. The entire subcortical area of the cerebral hemispheres with severe, dilatation of ventricles and cerebrospinal fluid(CSF) wits absent. There was parenchyma atrophy affecting chiefly in the white mater and the cerebral cortices, axon degeneration and necrosis and gitter cell infiltration in the whiter matter and the subependymal area. Mononuclear perivacular cuffing in the cerebrum and the pons was shown. Based on the radiological, gross and histopathological findings, this case was believed to have congenital hydrocephalus with nonsuppurative encephahitis. Possible etiology on the case is also discussed.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.44 no.4
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• pp.198-203
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• 2018

Dyke-Davidoff-Masson syndrome is a non-inherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. The present article highlights a case of a 12-year-old male child with additional clinical findings of $caf{\acute{e}}$-au-late pigmentation and ocular lipodermoid. This is the first case report of DykeDavidoff-Masson syndrome to describe oral manifestations, such as unilateral delayed eruption of teeth, hypoplasia, and taurodontism, which could be unique and characteristic of this condition. Oral health care providers and physicians should be aware of these oral observations as dental referrals could warrant early dental prophylactic care and can be useful in diagnosing the possible time of injury and type of Dyke-Davidoff-Masson syndrome.