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Clinical Heterogeneity in Korean Patients with Nemaline Myopathy
홍지만,김승민,선우일남,김세훈,김태승,심동석,최영철 연세대학교의과대학 2010 Yonsei medical journal Vol.51 No.2
Purpose: Nemaline myopathy (NM) is a clinical heterogeneous congenital myopathy characterized by the presence of subsarcolemmal or cytoplasmic rod-like structures that call nemaline bodies in the muscle fibers. The purpose of this study was to investigate the clinical diversity and pathological features of Korean patients with NM. Materials and Methods: Eight patients underwent analyses of clinical manifestations by a structured protocol. Diagnoses were established by a muscle biopsy. Results: Two patients had the typical congenital type, which exhibited neonatal hypotonia and delayed motor milestone, and five patients had the childhood onset type, which exhibited mild gait disturbance as a first symptom. One patient had the adult onset type, which showed acute respiratory failure. Limb weakness was proximal-dominant occurred in six patients. Hyporeflexia was observed in most patients. Elongated faces and high arched palates and feet were also observed. On light microscopy, the nemaline bodies were observed in type 1 and 2 fibers. All patients showed type 1 predominance and atrophy. In the two cases in which ultrastructural studies were performed, typical nemaline rods and disorganized myofibrillar apparatus were detected. Conclusion:In conclusion, the eight Korean patients in this study with NM shared common clinical expressions such as proximal limb weakness, reduced deep tendon reflex, and dysmorphic features. This study, however, showed that clinical heterogeneity ranged from typical congenital, mildly affected childhood to the adult onset form with acute respiratory failure. The pathological findings in this study were in accordance with those of other previous reports.
홍지만,최준영,박희영,허균 대한의학회 2008 Journal of Korean medical science Vol.23 No.6
We report a 43-yr-old man manifesting bacterial meningoencephalitis and multiple abscesses by Streptococcus pneumoniae. Serial magnetic resonance (MR) imagings and MR spectroscopy showed the evolution of multiple brain abscesses over 4 weeks: the enhanced rings became thicker and the dimension of whole lesions larger despite shrinkage of the ring-enhanced regions. These findings may be evidence of active inflammation working to sequestrate the lesion and protect the surrounding normal brain parenchyma from additional damage, even in the final stage of the brain abscess.
홍지만,정기영 대한내과학회 2006 The Korean Journal of Internal Medicine Vol.21 No.2
Severe complications such as meningoencephalitis are quite rare in the setting of mumps infection. We report here on a 24-year-old soldier with a past history of MMR vaccination who was admitted because of aseptic meningitis. His mental status rapidly deteriorated and swelling of both parotid glands was observed. The MR findings revealed discrete lesions in the brainstem and thalamus. He barely recovered within 2 months. Among the adults with failure to acquire immunity despite MMR vaccination, institutional crowding can lead to cases of potentially fatal CNS complication such as mumps meningoencephalitis
동시에 측정한 기저동맥과 중대뇌동맥의 이산화탄소 반응성: Power M-mode 도플러와 전-후 탐침 고정장치의 이용
홍지만,신동훈,허균,주인수,신상건,이승남 대한신경과학회 2007 대한신경과학회지 Vol.25 No.1
Background: Studies using a transcranial Doppler (TCD) to establish cerebral vasoreactivity (CVR) have mostly focused on the anterior circulation. The purpose of this study is not only to evaluate the feasibility of the power motion mode Doppler (PMD) with a probes fixating device, but also to simultaneously measure the CVR between the middle cerebral artery (MCA) and the basilar artery (BA) during hypercapnea simulated by the rebreathing technique. Methods: Twenty eight healthy volunteers were enrolled. Baseline hemodynamic values (heart rate, blood pressure) were measured while volunteers sat in a comfortable position for 5 minutes. The TCD was performed in two steps. First, velocities and spectra of the MCAs were simultaneously monitored. Then, the velocities and spectra of the MCA and BA were simultaneously monitored by a headset that included an anterior-posterior probes fixating device. The equation for CVR was ([maximum mean velocity baseline mean velocity]×100/baseline mean velocity). Results: Baseline mean velocities were revealed as follows: (64.0±13.7, 65.0±11.9 cm/s in right and left MCA; p>0.05; 67.3±12.2, -45.3±7.6 cm/s in dominant MCA and BA). CVR did not differ between the dominant MCA and the BA (46.1±12.1, 46.0±15.1%; p>0.05), nor between the right and left MCAs (46.9±15.2, 46.4±14.8%; p>0.05). There was a positive linear correlation between the CVR of the dominant MCA and that of the BA (r=0.856; p<0.001). Conclusions: PMD with a probes fixating device for accurate insonation is a useful tool for evaluating the relative CVR between the MCA and BA. Our study suggests that CVR values of the BA are similar to those of the MCA.
홍지만,신상건,허균,주인수 대한신경과학회 2007 대한신경과학회지 Vol.25 No.2
Background: In migraine studies, the cerebrovascular reactivity (CVR) using a transcranial Doppler (TCD) has been investigated to elucidate the nature and role of the vascular response. However, past studies have not comprised the posterior circulation including functionally important brainstem structures. The purpose of this study was to compare the simultaneous CVRs between the middle cerebral artery (MCA) and basilar artery (BA) in migraine patients with and without aura, by means of a power motion mode Doppler (PMD) with an anterior- posterior probes fixating device. Methods: Thirty-six consecutive patients with migranes [15 migraine patients with aura (MA) and 21 migraine patients without aura (MWA)] were compared with 29 healthy volunteers. CVR [(Vmax-Vbase)×100/Vbase] was evaluated by the re-breathing technique. TCD was performed as two steps. First, the velocities and spectra of the MCAs through both temporal windows were simultaneously monitored. Second, those were simultaneously monitored between MCA and BA. Result: There were no significant differences in age, sex, baseline hemodynamic values (blood pressure, heart rate), and those of the baseline mean flow velocity and CVR of TCD between the migraine patients and the controls. However, the CVR of the BA significantly differed between the MA and the MWA (39.4±13.7 vs 64.6±25.4%; p=0.001), among MA, MWA, and controls (39.4±13.7, 64.6±25.4, 45.6±14.9%; p<0.001). Conclusions: CVR of BA was entirely different according to migraine subtypes: with or without aura. Our study suggests that MWA and MWOA seem to be distinct disorders in terms of different vascular responses of the BA during the interictal period.
홍지만,김현숙,김원주,이경열 대한신경과학회 2004 대한신경과학회지 Vol.22 No.4
Cryptococcal meningitis is one of the common fungal infections of the central nervous system, usually developed in immunocompromised patients. Cerebral infarction has been reported as one of the late complications in cryptococcal meningitis. We report a case of cryptococcal meningitis, which initially presented with multiple cerebral infarctions of the bilateral basal ganglia and thalamus without the usual clinical signs of meningitis.
홍지만,박민수,전동철 대한신경과학회 2005 대한신경과학회지 Vol.23 No.5
With the exception of patients with epilepsy, transient splenial lesion of the corpus callosum (SCC) has been rarely reported. We investigated two young men with temporary encephalopathy. One had a staphylococcal infection, the other had a viral infection. The brain MRI findings of these patients showed a transient focal lesion in the splenium of the corpus callosum. Transient splenial lesions of the corpus callosum might be a non-specific end point of a different disease process leading to cytotoxic edema or osmotic demyelination of the brain.