Unilateral Hypoglossal Neuropathy in Multifocal Acquired Demyelinating Sensory and Motor Neuropathy: Differential Diagnosis of Motor Neuron Disease

Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is a peripheral neuropathy characterized by multifocal weakness and associated sensory impairment. MADSAM is associated with multifocal persistent conduction block and other signs of demyelination. The incidence of cranial nerve involvement in MADSAM was recently reported to be approximately 15%. However, reports of hypoglossal neuropathy occurring in MADSAM are rare. Unilateral hypoglossal neuropathy in MADSAM is usually misdiagnosed as motor neuron disease. We report a patient with MADSAM presenting with tongue hemiatrophy.

Simultaneous Occurrence of a Splenial Lesion and Downbeat Nystagmus Due to Lithium Neurotoxicity

No abstract available.

Bilateral Lesions in the Posterior Limb of Internal Capsule Attributed to Combination Therapy of Isoniazid and Rifampin

Transient isoniazid-induced brain lesions have rarely been reported. The lesions were in the dentate nucleus of cerebellum and thalamus. Meanwhile, the neurotoxicity of rifampin has not been reported evidently. We observed bilateral lesions in the internal capsule in a young woman after taking a combination of isoniazid and rifampin. She transiently suffered numbness in both hands, dysarthria, and left side motor weakness while taking the medication. Isoniazid may induce structural lesions in various brain areas including the internal capsule.

Recurrent Ischemic Strokes with Progression of Middle Cerebral Artery Stenosis during HIV Treatment

Human immunodeficiency virus (HIV) infection can result in ischemic stroke via several mechanisms, including opportunistic infection, vasculopathy, cardioembolism, and coagulopathy. HIV-vasculopathy is related to endothelial dysfunction, stenosis and aneurysm formation, infectious vasculitis, dissection and accelerated atherosclerosis during highly active antiretroviral therapy (HAART). We represent a case of HIV infection manifested as an acute ischemic stroke attack. After 4 months during HAART, our patient experienced a recurrent ischemic stroke with progression of middle cerebral artery stenosis.

A giant retroperitoneal lymphangioma in a patient with neurofibromatosis type 1 / 대한외과학회지

Neurofibromatosis type 1 (NF-1) is a genetically inherited disorder that may cause skin abnormalities and tumors that form on nerve tissues. These tumors can be small or large and can occur anywhere in the body, including the brain, spinal cord, or other peripheral nerves. Retroperitoneal lymphangiomas are very rare benign malformations of the lymphatic system. About 95% lymphangiomas occur in the skin and the subcutaneous tissues of the head, neck and axillary region and the remaining 5% appear in other parts of the body such as lungs, pleura, pericardium, liver, gallbladder, kidney, and the mesentery. Herein, we report the case of a giant retroperitoneal lymphangioma in a patient with NF-1 with a review of the literature.

Hyponatremia Induced Coma during Risperidone Treatment in 2 Cases of Schizophrenia / 대한정신약물학회지

Antipyschotics have been frequently reported being associated with hyponatremia. We have experienced two cases of severe hyponatremia with neurological symptoms in patients with acute schizophrenia who were treated with risperidone. Case 1 was a 33-year-old female who developed hyponatremia (Na+114 mEq/L) at 17 days after initiation of treatment with risperidone. Case 2 was a 41-year-old female who developed hyponatremia (Na+107 mEq/L) 5 months after treatment with risperidone. In both cases, polydipsia was not obvious. The exact cause of hyponatremia was uncertain, but we suggest that syndrome of inappropriate secretion of antidiuretic hormone due to risperidone may be the mechanism of hyponatremia in both cases. Although there have been many recent studies about the pathophysiology of hyponatremia among psychiatric patients, the exact mechanism remains unclear. There have been many case reports in the literature of hyponatremia associated with antipsychotics. We suggest that risperidone may be a potential drug that can cause hyponatremia and lead to life-threatening neurological symptoms in the early stage treatment, and that clinicians should monitor patients on a regular basis.

Cardiac tamponade in a patient with lupus nephritis / 대한내과학회지

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that involves many different organ systems, and this illness exhibits a wide spectrum of clinical manifestations. The renal involvement in SLE is called lupus nephritis and 50~80% of patients with SLE suffer from lupus nephritis. Although pericarditis or pericardial effusion is a common cardiac manifestation of SLE, cardiac tamponade is rare. Cardiac tamponade may be fatal if it is not recognized quickly and treated promptly. We report a case of cardiac tamponade associated with lupus nephritis. Treatment consisted of pericardiocentesis and high-dose corticosteroid.

Continuous ambulatory peritoneal dialysis in a patient with diabetic chronic renal failure and hemophilia / 대한내과학회지

Long-term dialysis is undertaken infrequently in patients with hemophilia and chronic renal failure, and there are no definite guidelines for the treatment of end-stage renal disease in hemophiliacs. We report a case of peritoneal dialysis in a hemophiliac with diabetic chronic renal failure. A 46-year-old man with severe hemophilia was admitted to our hospital for decreased visual acuity and the management of uremia. After vitrectomy, he was started on hemodialysis to treat his uremic bleeding diathesis. To avoid bleeding through a vascular access site, peritoneal dialysis therapy for long-term treatment was proposed. A surgical peritoneal catheter was introduced with simultaneous factor VIII administration. In the seven subsequent months, no episodes of intraperitoneal or retroperitoneal hemorrhage have been observed. Continuous ambulatory peritoneal dialysis may be a relatively safe dialysis modality without danger of bleeding in hemophiliacs.

Constipation is Closely Associated with Depression in Patients with End-Stage Renal Disease Undergoing Hemodialysis or Peritoneal Dialysis / 대한신장학회지

PURPOSE: Constipation is a frequent complaint among dialysis patients. However, factors that contribute to constipation in these patients have not been evaluated rigorously. The aim of study was to assess the prevalence and factors that contribute to constipation in patients with End-Stage Renal Disease (ESRD) undergoing hemodialysis (HD) or peritoneal dialysis (PD). METHODS: Patients undergoing HD or PD for more than six months in the six dialysis centers were asked to complete a self-administered questionnaire that is designed to assess constipation by Rome- III criteria. Beck depression inventory (BDI) were assessed. A total of 146 patients (HD 91, PD 55) completed the study. RESULTS: The prevalence of constipation was 33% in 91 HD patients and 31% in 55 PD patients. Prevalence of constipation was 32% and did not differ by dialysis mode. Older age, unemployed state, high cumulative illness rating scale and high BDI were associated with constipation. In multivariate analysis, BDI was an independent factor associated with constipation. The prevalence of constipation was 18% and 50% for patients with BDI or =15, respectively. The odd ratio for constipation in patients with BDI > or =15 was 3.4 (95% CI, 1.4-8.1). CONCLUSION: Careful psychogenic attention must be paid to ESRD patients with constipation.

A Case of Concurrent Pneumocystis carinii Pneumonia and CMV Pneumonia in a SLE Patient with Mycophenolate Mofetil / 대한신장학회지

A 36-year-old woman developed concurrent Pneumocystis carinii pneumonia (PCP) and cytomegalovirus (CMV) pneumonia. She was diagnosed as lupus nephritis two years ago and was on immunosuppressive therapy with prednisolone and mycophenolate mofetil. She developed dyspnea with moderate hypoxemia. Chest X-ray and HRCT showed diffuse interstitial infiltration and ground glass opacity. The diagnosis of concurrent PCP and CMV was performed by the virus culture and PCP PCR of bronchoalveolar lavage fluid. She recovered following treatment with trimethoprim-sulfamethoxazole, prednisolone, gancyclovir for three weeks.

Metronidazole-Induced Toxic Encephalopathy in A Hemodialysis Patient: A Case Report / 대한신장학회지

Metronidazole, one of the mainstay drugs for the treatment of anaerobic infections and protozoal infections, may produce some serious neurological side effects. Seizure, peripheral neuropathy, dizziness, vertigo, ataxia, confusion, irritability, headache and tremor have been reported among patients receiving metronidazole, particularly among those receiving high dose of the drug. However, no specific dose adjustment is recommended for patients with renal or hepatic dysfunction receiving metronidazole because the accumulation of the drug and its metabolites can occur in terms of severity of illness and patient tolerability. Although metronidazole is significantly removed by hemodialysis, no specific dose recommendations are made for this patient population. Recently, we experienced metronidazole-induced encephalopathy in a patient with end-stage renal disease, which was diagnosed by brain magnetic resonance imaging (MRI). Herein, we report a case with a review of literature.

The Changes of Clinical Markers, beta2-microglobulin and Oxidized LDL, after Converting to On-line Hemodiafiltration from High-flux Hemodialysis / 대한신장학회지

PURPOSE: Hemodiafiltration with the on-line regeneration of ultrafiltrate (on-line hemodiafiltration, OL- HDF) provides better clearance of both small and large solutes than high-flux hemodialysis (HFHD) and may reduce inflammation and oxidative stress. The purpose of this observational study was to ascertain whether OL-HDF improves clinical markers after switching from HFHD to OL-HDF in patients with end-stage renal disease. METHODS: Fourteen stable patients on HFHD for at least 6 months were switched to OL-HDF. We observed changes in subjective symptoms, the levels of hemoglobin, erythropoietin dose, Kt/V, URR, beta 2-microglobulin, plasma oxidized LDL (OxLDL) and CRP for twelve-months period. RESULTS: There were improvements in subjective symptoms including fatigue, anorexia, insomnia and itching sensation (p<0.05). There were significant increases in Kt/V (1.63+/-0.17 vs. 1.77+/-0.24, p< 0.05) and URR (75.1+/-3.2 vs. 77.2+/-3.9%, p<0.05). Even though erythropoietin dose was not changed, hemoglobin level was increased (10.6+/-1.3 vs. 11.4+/-1.0, p<0.05). Beta 2-microglobulin clearance was significantly increased (17.7+/-4.4 vs. 21.9+/-6.1 mL/min, p<0.05). However, the pre-dialysis level of beta 2-microglobulin was not significantly reduced. The pre-dialysis level of CRP was not changed. OxLDL reduction ratio was not changed. On the other hand, the pre-dialysis plasma level of OxLDL was significantly reduced (32.36+/-6.03 vs. 26.05+/-6.00 U/L, p<0.05). CONCLUSION: OL-HDF improved several subjective symptoms, anemia, the clearance of small solute, beta 2-microglobulin level and reduced plasma OxLDL after switching from HFHD. Less OxLDL may be generated in OL-HDF compared with HFHD.

A Case of End-Stage Renal Disease (ESRD) from Reflux Nephropathy in a patient with Situs Inversus and Duplicated Ureter / 대한신장학회지

Situs inversus is a rare congenital anomaly that occurs in adults at the rate of 1:5,000-1:10,000. In the total form, the thoracic organs, as well as the abdominal organs, are completely reversed in a "mirror image" of their normal arrangements. Ureteral duplication is the most common ureteral anomaly founded incidentally. However, there is a higher incidence of duplication in cases of urinary tract infection, and it may be associated with upper urinary tract stasis, obstruction, or reflux. But ureteral duplication has no relation to situs inversus. Vesicoureteral reflux (VUR) is the most common disorder usually detected during radiologic evaluation of children with urinary tract infection. Complications such as renal scarring, reflux nephropathy, chronic renal failure and hypertension are well known in patients with VUR. Reflux nephropathy is responsible for about 10% of all cases of treated ESRD and is the most common case of ESRD in children. Thus, if VUR exists, early diagnosis and intensive treatment is necessary. Herein, we present a case of reflux nephropathy related ESRD in a 41-year-old woman with total situs inversus and duplicated ureter.

Tuberculous Liver Abscess in a Patient on Continuous Ambulatory Peritoneal Dialysis / 대한신장학회지

The incidence of tuberculosis (TB) in patients on regular hemodialysis and continuous ambulatory peritoneal dialysis (CAPD) patients is higher than that of general population. But, tuberculous liver abscess is rare even in endemic area and is found in 0.34% of hepatic tuberculosis. Extrapulmonary tuberculosis shows atypical presentation, and prompt diagnosis and treatment is important for prognosis of the patient. We report a case of CAPD patient with primary tuberculous liver abscess. The patient was treated successfully with antituberculous therapy after the diagnosis was established by histopathologic examination from the specimen obtained by ultrasound-guided percutaneous biopsy of the liver.

A Case of Concomitant Interstitial Nephritis by BK Virus with Acute Rejection in a Renal Allograft Recipient / 대한신장학회잡지

Polyomavirus BK viral allograft nephritis is a great challenge in posttransplant management and graft survival because of difficulty in diagnosing and treatment. Initial treatment usually involves reducing immunosuppressive medications. However if concomitant acute rejection exist, it is more challenging in managing these patients, because acute rejection requires increase in immunosuppression. We present a case of a 35-year-old man who developed BK viral allograft nephritis and concomitant acute rejection 3 months after transplantation. BK viral allograft nephritis was missed in diagnosis and only pulse steroids for anti-rejection therapy was done. Initially, renal function was improved, but 4 months later, he presented with deterioration in renal function. Second renal biopsy showed BK allograft nephritis without rejection. BK viral DNA in plasma by PCR and urinary decoy cell were also positive. Reduction in immunosuppression by discontinuing mycophenolate mofetil stabilized the deterioration in renal function, however it failed to clear viremia.

Comparison of Safety and Efficiency of Hemodialysis Using Heparin-bound Hemophan with those of Saline Flushing Hemodialysis and Hemodialysis Using Low Dose Heparin in Patients at Risk of Bleeding / 대한신장학회잡지

OBJECTIVE: Although hemodialysis using heparin bound Hemophan (HBH-HD) has been reported to be a possible modality in patients at risk of bleeding, the efficiency and safety of HBH-HD is not certain. Therefore, we prospectively compared the safety and efficiency of HBH-HD with those of saline flushing HD (SF-HD) and HD using low dose heparin (LDH-HD) in 13 HD patients at risk of bleeding in a cross-over design. METHODS: The safety and efficiency were evaluated by measuring activated partial prothrombin time (aPTT) before and during HD, hemostasis time after needle removal, total blood compartment volume (TBCV) loss of dialyzer, urea clearance (K) and Kt/V. RESULTS: There was no difference in compression time needed to achieve hemostasis at puncture site after needle removal between HBH-HD, SF-HD and LDH-HD. During HBH-HD, there was a slight increase in aPTT at 15 min (50.6+/-4.5 sec), compared to predialysis levels (40.9+/-4.7 sec). In this cross- over study, aPTT during dialysis session was markedly higher in LDH-HD than those in HBH-HD or SF-HD (p<0.05). The loss of TBCV of the dialyzer was greater in SF-HD than HBH-HD or LDH-HD (17.4+/-1.9% vs. 12.4+/-1.4% vs. 10.1+/-1.8%). However, there was no difference in K (212.0+/-30.7 vs. 217.2+/-36.9 vs. 221.6+/- 29.5 mL/min) and Kt/V (1.22+/-0.12 vs. 1.24+/-0.16 vs. 1.26+/-0.18). CONCLUSION: We concluded that the safety and efficiency of HBH-HD are not different compared to SF-HD or LDH-HD and HBH-HD could an alternative hemodialysis method in patients at risk of bleeding.

Mycophenolate Mofetil Therapy in Severe Membranous Nephropathy and Diffuse Proliferative Lupus Nephritis: Clinical Observation / 대한신장학회잡지

OBJECTIVE: Although cyclophosphamide (CYC) is effective for the treatment of diffuse proliferative lupus nephritis (DPLN) and severe membranous nephropathy (MN), it has serious adverse effects. Therefore, we evaluated our clinical observations with mycophenolate mofetil (MMF) for empirical treatment of DPLN and severe MN. METHODS: Seventeen patients with biopsy proven severe MN (n=8) and DPLN (n=9) received MMF for > or = 6 months as primary treatment (n=9) or subsequent maintenance therapy after CYC treatment (n=8). Treatment outcome was evaluated by random urine protein/creatinine ratio (UP/Cr) and serum creatinine (sCr) at the start and at 12 months and compared by the Wilcoxon signed-rank test. RESULTS: Overall, the mean (+/-SD) UP/Cr decreased in both MN (6.48+/-3.03 vs. 1.31+/-1.22, p= 0.016) and DPLN (3.77+/-2.34 Vs 0.83+/-0.53, p=0.043) patients. No significant change in serum Cr was detected in both MN and DPLN patients. Adverse events included nausea/abdominal discomfort (n=1) and menstrual irregularity (n=1). CONCLUSION: Short term empirical treatment with MMF in the majority of patients with severe MN and DPLN was well tolerated and effective in decrease of proteinuria and stabilization of renal function. Controlled clinical trials are necessary to define the role of MMF in the treatment of severe MN and DPLN.

Effect of High Dose (10, 000 IU) Epoetin Alfa (Espogen(R)) Therapy in CAPD Patients / 대한신장학회잡지

BACKGROUND: Recombinant human erythropoietin (rHuEPO) is an established treatment for renal anemia. We aimed to determine that high dose subcutaneous epoetin alfa is as efficient and safe as usual dose for treating anemia in peritoneal dialysis patient. METHODS: Twenty four patients on CAPD were randomly assigned to either 10, 000 IU (high dose group, n=12) or 4, 000 IU (usual dose group, n=12) epoetin alfa regimen with variable interval for 24 weeks. If hematocrit was out of range (30-39%), the interval was changed within 50% of previous interval. RESULTS: Mean hemoglobin levels at randomization and after 12 weeks and 24 weeks were 11.4+/-1.3, 11.3+/-1.1, and 11.6+/-1.2 g/dL in high dose group compared with 10.8+/-0.8, 11.5+/-1.1, and 10.9+/-1.2 g/dL in usual dose group (p<0.05). The mean weekly epoetin alfa dosages at randomization and after 12 and 24 weeks were 93.2+/-45.3, 95.5+/-33.6, and 102.5+/-43.6 IU/kg in high dose group compared with 78.8+/-29.4, 75.9+/-20.6 and 75.5+/-39.7 IU/kg in usual dose group (p<0.05). But, interval in high dose group was two times as longer as usual dose group. Adverse events were generally mild and transient CONCLUSION: This study demonstrates that epoetin alfa 10, 000 IU is as efficient and safe as 4, 000 IU with similar weekly dose in CAPD patients. epoetin alfa 10, 000 IU administration reduces frequency of injections about one half.

Clinical Manifestations of Acute Renal Failure with Loin Pain Developed after the Anaerobic Exercise / 대한신장학회잡지

BACKGROUND: Rhabdomyolysis is a well known cause of the exercise induced acute renal failure (ARF), but the patch renal vasoconstriction with severe loin pain which developed after the anaerobic exercise is not. Although there are a few cases about ARF resulted from the patchy renal vasoconstriction in Korea, there are only a few reports about comparisons of their clinical manifestations. METHODS: Eight patients of ARF with severe loin pain after the exercise, were admitted to Chungbuk national university hospital from April 1994 to March 2004. For all patients, we obtained basic clinical findings and laboratory studies, and performed an enhanced computed tomography (CT) initially and delayed CT without contrast media at least 6 hours after. RESULTS: All the patients were previously healthy young men. The main symptom was loin pain in five patients and all patients experienced the anaerobic exercise before. Six patients took analgesics and fever was observed in seven patients. There was no marked elevation of creatinine phosphokinase (CPK). The initial mean creatinine (Cr) was 3.50+/-1.49 mg/dL and the maximum Cr was 8.8 mg/dL. All the patients fully recovered their renal function. We observed the typical patchy wedge-shaped contrast enhancement CT findings in seven patients. CONCLUSION: Differential diagnosis between rhabdomyolysis and the patchy renal vasoconstriction in newly developed ARF after exercise is important. Our eight patients showed typical clinical manifestations. In cases which implicate the pathy renal vasoconstriction by their typical clinical findings, the enhanced CT and the delayed postcontrast CT are helpful for diagnosis.

A Case of Sclerosing Peritonitis Successfully Treated with Corticosteroid Therapy / 대한신장학회잡지

Sclerosing peritonitis is a rare but fatal complication of peritoneal dialysis (PD). Management of sclerosing peritonitis includes cessation of PD, total parenteral nutrition, and surgery. Recently, a few reports have indicated immunosuppression might be beneficial in sclerosing peritonitis. In these reports, all of patients had the combination therapy of steroid and immunosuppressant. A 37-year old man develped sclerosing peritonitis 3 months after switching from PD to hemodialysis because of uncontrolled peritonitis. An abdominal computed tomography (CT) scan demonstrated massive ascites with multilocuated fluid collection and extensive enhancement of the peritoneum. A peritoneal biopsy showed proliferation of fibrous collagenous tissue with infiltration of lymphocytes. We started corticosteroid for one month. A follow-up CT scan showed complete resolution with absence of peritoneum thickness and fluid collection 16 months after corticosteroid therapy. The patient currently remains free of symptoms in an outpatient hemodialysis unit. To our knowledge, this is the first case of sclerosing peritonitis successfully treated with corticosteroid therapy alone in Korea.