• Clinical and Experimental Pediatrics
• /
• 제53권7호
• /
• pp.759-765
• /
• 2010

Purpose: This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts. Methods: Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain magnetic resonance imaging (MRI) in 29 patients. The clinical, hormonal, and imaging features were reviewed retrospectively. Results: The clinical presentation of symptomatic patients was as follows: headache (65%), endocrinopathy (61%), and visual disturbance (19%). Endocrinopathy included central precocious puberty (18%), diabetes insipidus (14%), general weakness (11%), and decreased growth velocity (7%). After surgery, hyperprolactinemia resolved in all patients, but growth hormone insufficiency, hypothyroidism, and diabetes insipidus did not improve. Pituitary insufficiency except gonadotropin abnormality correlated significantly with severe headache, visual disturbance, general weakness, and cystic size. Suprasellar extension of cysts and high signals in the T2-weighted image on brain MRI were related to hypothyroidism, hypocortisolism, and diabetes insipidus. Multivariable linear regression analysis showed that only general weakness was a risk factor for pituitary insufficiency ($R^2$=0.549). Conclusion: General weakness is a risk factor for pituitary insufficiency in patients with Rathke's cleft cysts. When a patient with a Rathke's cleft cyst complains of general weakness, the clinician should evaluate pituitary function and consider surgical treatment.

• Annals of Clinical Neurophysiology
• /
• 제6권1호
• /
• pp.14-19
• /
• 2004

Backgrounds and objectives: POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is the rare cause of polyneuropathy. Although the polyneuropathy is essential for the diagnosis of the disease, the pattern of electrodiagnostic abnormalities has not been characterized in detail. The purpose of this study was to elucidate the features of nerve conduction abnormalities in POEMS syndrome. Methods: We reviewed the medical records and nerve conduction studies (NCS) of 12 consecutive patients with POEMS. Results: A total of 68 motor and 46 sensory nerves were examined. Compound muscle action potentials (CMAPs) and sensory nerve action potentials were abnormally attenuated or not elicited in majority of motor and sensory nerves (80.88% in motor, and 82.6% in sensory nerves). Frequency of the nerves with no potential was significantly higher in lower limbs than in upper limbs (p<0.01 in both motor and sensory nerves), and CMAP amplitude was more reduced in lower limbs than in upper limbs (p<0.01). Conduction slowing was very frequently observed with 95% and 76% of motor and sensory nerves, respectively, having the abnormally reduced values of conduction velocity. Distal motor latencies were abnormally prolonged in 75% of motor nerves, and terminal latency indices were significantly higher in patients than in normal controls (p < 0.05). Conduction block was observed only in 5% of motor nerves. Conclusions: NCS in POEMS syndrome showed characteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lower limbs, and more predominantly in the intermediate nerve segments than in the distal portions. The recognition of these characteristic patterns may be helpful in early diagnosis of polyneuropathy in POEMS syndrome.

• 정신신체의학
• /
• 제4권1호
• /
• pp.98-108
• /
• 1996

Korean women are now living almost 1/3 of their life after menopause. Sex-steroid hormone deficiency adversely affect various fields of physical & mental activity and quality of life during this period. Therefore, replacement of deficient hormone is thought to be natural therapeutic modality. Postmenopausal syndrome is recently redefined as an endocrinopathy with both short-and long-term sequelae, as a result of cessation of ovarian function. Hormone replacement therapy taken at or near time of menopause alleviates shot-term acute menopausal symptoms such as vasomotor disturbances and psychological problems. HRT also beneficially affects some of intermediate symptoms such as urogenital atrophy and cutaneous problems. The major benefits of long-term use were reductions in risk of long-term sequelae, that is, total fracture by 50-60%, cardiovascular disease by 50% and cerebrovascular disease by 30-40%, respectively. In addition, HRT may also positively influence Alzheimer's disease, reduce the colorectal cancer risk and increase longevity of the life. In conclusion, all postmenopausal women should consider preventive HRT when there are no contraindications.

• Nuclear Medicine and Molecular Imaging
• /
• 제41권1호
• /
• pp.66-67
• /
• 2007

POEMS syndrome is a rare disorder, also known as Crow-Fukase, PEP or Takatsuki syndrome. The acronym, POEMS, represents polyneuropathy, organomegaly, endocrinopathy, M protein and skin change. However, there are associated features not included in the acronym such as sclerotic bone lesions, Castleman disease, papilledema, thromobocytosis, peripheral edema, ascites, effusion, polycythemia, fatigue and clubbing. In most cases, osseous lesions in POEMS syndrome present as an isolated sclerotic deposit and that reveal as osteosclerotic myeloma. Several cases of $^{18}F-FDG$ PET in multiple myeloma involvements were reported, but there was no previous literature that reported FDG PET findings in POEMS syndrome. We describe here a 66-year-old patient with POEMS syndrome who underwent $^{18}F-FDG$ PET/CT image.

• Clinical and Experimental Pediatrics
• /
• 제45권9호
• /
• pp.1165-1169
• /
• 2002

저자들은 근 경련에 의한 근육통과 전신성 탈모증으로 내원하여 Satoyoshi 증후군으로 진단된 1례를 경험하였기에 보고하는 바이며, 근 경련에 탈모증이 동반된 환아의 진단에 있어 Satoyoshi 증후군을 고려해야 할 것으로 사료된다.

• 대한수의학회지
• /
• 제56권3호
• /
• pp.161-166
• /
• 2016

Hyperadrenocorticism (HAC) is a common endocrinopathy among dogs that causes multisystemic signs. This study was conducted to evaluate cardiocirculatory, biochemical, and hemostatic parameters in dogs with HAC at diagnosis, in addition to verifying whether abnormal parameters could be controlled by initial treatment with trilostane. Fifteen dogs with HAC were assessed by systolic blood pressure measurement, electrocardiography, Doppler echocardiography, serum concentration of troponin I, and biochemical and hemostatic profile at diagnosis and after trilostane therapy. Unlike biochemical parameters, hemostatic and cardiocirculatory parameters were not significantly influenced by the onset of treatment. The authors believe that clinical treatment with trilostane for 3 to 4 months might not be sufficient for the stabilization of cardiocirculatory abnormalities such as hypertension. Therefore, dogs with HAC must receive cardiocirculatory monitoring at diagnosis and during drug treatment.

• Annals of Clinical Neurophysiology
• /
• 제5권2호
• /
• pp.210-213
• /
• 2003

POEMS syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein(M-protein), and skin change. Recently we have had the opportunity to attend one patient with clinical features similar to this syndrome. He was a 46-year-old man who had a progressive polyneuropathy, swallowing difficulty, hepatosplenomegaly, hypothyroidism, IgA ${\lambda}type$ monoclonal gammapathy, specific skin change and ascites. His symptoms such as low extrimity pain and weakness, swallowing difficulty were improved by high-dose 7S-IgG. Thus, we report a case with a review of the literature.

• Journal of Genetic Medicine
• /
• 제17권1호
• /
• pp.1-10
• /
• 2020

Polycystic ovarian syndrome (PCOS) is the most common endocrine disorder in women, which is characterized by the oligo/anovulation, hyperandrogenism (HA) and polycystic ovarian morphology which are diagnostic criteria. PCOS has diverse clinical aspects in addition to those diagnostic criteria including increased risk for cardiovascular diseases, metabolic syndrome, dyslipidemia, type 2 diabetes and impaired fertility. Because of the heterogeneity of the disease, the pathogenesis of the disease has not been elucidated yet. Therefore, there is no cure for the endocrinopathy. HA and insulin resistance (IR) has been considered two major pillars of the pathogenesis of PCOS. Recent advances in animal studies revealed the critical role of neuroendocrine abnormalities in developing PCOS. Several pathways related to neuroendocrine origin have been investigated such as hypothalamus pituitary ovarian axis, hypothalamus pituitary adrenal axis and hypothalamus pituitary adipose axis. This review summarizes the current knowledge about the role of HA and IR in developing PCOS. In addition, we review the results of recent genome wide association studies for PCOS. This new perspective improves our understanding of the role of neuroendocrine origins in PCOS and suggest a novel potential therapeutic target for the treatment of PCOS.

• 대한신경과학회지
• /
• 제36권4호
• /
• pp.350-353
• /
• 2018

Ischemic stroke caused by the cerebral vasculopathy is a rare complication of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. We present a case of recurrent ischemic strokes caused by cerebral vasculopathy in a patient with POEMS syndrome. A 34-year-old man presented with gait disturbance and dizziness. Brain magnetic resonance imaging demonstrated acute ischemic stroke in the middle cerebral artery-anterior cerebral artery (MCA-ACA) border zones of bilateral hemispheres. Repeated angiographic studies showed progressive worsening of the left distal internal carotid artery, ACA, and MCA stenoses, along with sustained steno-occlusion of right MCA.

• Journal of Yeungnam Medical Science
• /
• 제32권2호
• /
• pp.122-126
• /
• 2015

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a monoclonal plasma cell disorder. Patients with POEMS syndrome also have various clinical manifestations including generalized edema, pleural effusion, ascites, papilledema, and sclerotic bone lesions. These manifestations can lead to a misdiagnosis or delayed diagnosis. We recently experienced a 51-year-old male patient with POEMS syndrome whose sclerotic bone lesion was misdiagnosed as malignant bone metastasis of papillary thyroid carcinoma. We reassessed the patient and found polyneuropathy, hepatosplenomegaly, hypothyroidism, partial hypopituitarism, immunoglobulin G lambda-type monoclonal gammopathy, hypertrichosis, ascites, and multiple sclerotic bone lesions, all of which led us to a diagnosis of POEMS syndrome. Treatment with thalidomide and dexamethasone resulted in clinical and radiological improvement. The patient has remained in remission after peripheral blood stem cell transplantation.