Ghrelin is a novel growth hormone (GH)releasing peptide, isolated from the stomach, which has been identified as an endogenous ligand for GH secretagogue receptor. The discovery of ghrelin indicates that the release of GH from the pituitary might be regulated not only by hypothalamic GHreleasing hormone, but also by ghrelin derived from the stomach. This peptide also stimulates food intake and induces adiposity through GHindependent mechanisms. In addition, ghrelin acts directly on the central nervous system to decrease sympathetic nerve activity. Thus, ghrelin plays important roles for maintaining GH release and energy homeostasis. Repeated administration of ghrelin improves body composition, muscle wasting, functional capacity, and sympathetic augumentation in cachectic patients with heart failure or chronic obstructive pulmonary disease. These results suggest that ghrelin has anticachectic effects through GHdependent and independent mechanisms. Thus, administration of ghrelin may be a new therapeutic strategy for the treatment of cardiopulmonaryassociated cachexia.
Objective: Gluten sensitivity is associated with multiple neurological abnormalities including gluten ataxia, motor neuron diseaselike neuropathy, small fiber type neuropathy, cognitive impairment, and even parkinsonism. We investigated whether or not gluten sensitivity is involved in Japanese patients with idiopathic cerebellar ataxia with extracerebellar presentation. Patients or Materials: Fourteen patients with idiopathic cerebellar ataxia with extracerebellar presentation (autonomic instability, parkinsonism, or pyramidal dysfunction in varying combinations) were screened for antigliadin antibodies (AGA) to analyze for the presence or absence of gluten sensitivity. Patients with typical MR findings of multiple system atrophy of the cerebellar type were excluded. As disease controls without cerebellar ataxia, 9 patients with Parkinsons disease and 18 patients with amyotrophic lateral sclerosis were screened for AGA. Fortyseven normal controls were also screened for AGA. Results: We found a high prevalence of AGA in 5 (36%) of 14 cerebellar ataxia patients, but in only 1 (4%) of 27 disease controls without cerebellar ataxia (odds ratio, 14.4; 95% CI, 1.41147; p<0.05) and in only 1 (2%) of 47 normal controls (odds ratio, 25.6; 95% CI, 2.66246; p<0.001). Among the cerebellar ataxia patients, atypical features such as sensorimotor neuropathy and/or mild cognitive impairment were more prevalent in the AGApositive group (60%) than in the AGAnegative group (0%). In one of the ataxic patients with AGA, a glutenfree diet had positive effects on neurological symptoms and nutritional status. Conclusion: Gluten sensitivity is involved in at least some of the unexplained neurological symptoms of Japanese patients with adultonset, sporadic cerebellar ataxia.
Objective: To clarify the clinical significance of subclinical rhythmic EEG discharge of adults' (SREDA) by analyzing characteristics of SREDA and the outcome of patients based on retrospective analysis of EEG data base. Methods: EEGs were recorded soon after the onset of patient's initial symptoms and repeatedly recorded at various intervals of 23 months in all 4 patients. Neurological findings, MRI and SPECT were also investigated. Subjects: Out of 340 consecutive inpatient population who had EEGs, 4 patients (1.2%) showed SREDA. They had a diagnosis of syncope, transient global amnesia, generalized tonicclonic seizure and right temporal lobe epilepsy for each. Results: There was no consistent abnormality in the brain MRI, CT or SPECT among the 4 patients. The acute and transient symptoms disappeared and did not recur within the followup period of 28 months in any patient. In 2 patients SREDA disappeared in the followup EEG taken 714 days after the first EEG showing SREDA. In the other 2 patients, the followup EEGs taken 5 days after the first EEG with SREDA when clinical symptoms disappeared showed less frequent occurrence of SREDA. Conclusion: Being different from the previous reports suggesting the relation with cardiogenic insults or persistent ischemic abnormality, SREDA can occur in patients with various acute brain dysfunctions followed by a favorable clinical outcome.
A 64yearold man was admitted for alithiasic cholecystitis. Necrotizing vasculitis was detected in a gallbladder obtained at the cholecystectomy. Slight elevation of transaminases, HBe antigens and hepatitis BDNA (HBVDNA) were detected in the patient. Intrahepatic necrotizing vasculitis was also detected in the liver biopsy specimen, and he also suffered from peripheral neuropathy of suddenly onset. Based on the diagnosis of hepatitis Brelated polyarteritis nodosa, lamivudine was initially administered, followed by plasmapheresis and glucocorticoid steroid therapy. These treatments brought satisfactory improvement of polyarteritis nodosa without exacerbation of liver function.
Accidental or deliberate ingestion of bromate solution has been reported in pediatric as well as adult cases; however there have been no reports of such intoxication in the elderly. We report a 78yearold woman who suffered severe acute renal failure due to the accidental ingestion of sodium bromate solution. The patient was successfully treated with hemodialysis therapy and renal function recovered without hearing loss. This case suggests that emergency therapeutic measures, including hemodialysis, should be taken as soon as possible, and the rapid removal of bromate is essential to prevent severe intoxication and its sequelae. To the best of our knowledge this is the first report of an elderly patient that demonstrates the clinical benefit of hemodialysis therapy for bromate intoxication.
We report a severe alcoholic hepatitis (SAH) patient with an extremely high WBC count, high serum bilirubin and low prothrombin time (PT) successfully treated with granulocytapheresis. After neutrophilelastase inhibitor failed to reduce WBC count, methylprednisolone pulse therapy was performed. However, WBC count continued to be elevated to 97,190 /μl (neutrophils 97.0%) despite improvement of total bilirubin and PT. After 5 sessions of granulocytapheresis and ulinastatin administration, increased serum IL6, IL8, neutrophilelastase and WBC count gradually decreased. We could conclude that granulocytapheresis and ulinastatin can be very effective in reducing cytokines and neutrophilelastase, and in improving the general status of SAH patients.
Myasthenic symptoms and the echocardiographic findings of dilated cardiomyopathy are very rare in primary AL amyloidosis. We report a 59yearold man with dyspnea on effort and weakness after exercise. His electrocardiogram showed ischemic heart disease and echocardiography indicated dilated cardiomyopathy. Muscle biopsy revealed amyloidosis with deposits of lambda light chainderived amyloid within the vessel wall. Treatment with PGE1 resulted in improvement of the myasthenic symptoms. This patient indicates that myasthenic symptoms and dilated cardiomyopathy would be a unique syndrome associated with systemic AL amyloidosis involving mainly the small vessels, i.e., AL amyloid angiopathy, in the skeletal muscles and myocardium vessels.
We report three elderly men with high serum IgG4 concentrations and multiple lymphoplasmacytic inflammation of the salivary glands, lymph nodes, pancreas, and renal interstitium. The infiltrating plasma cells had strong immunoreactivity for IgG4, even in patients without pancreatic lesions. These cases show that IgG4 is associated not only with autoimmune pancreatitis, but also with other systemic lymphoplasmacytic disease.
Nontuberculous mycobacterial pulmonary infection is a rare cause of a solitary pulmonary nodule. All previously reported cases were caused by Mycobacterium avium complex, and a solitary pulmonary nodule caused by other NTM species has been very rarely reported. We describe the first case of Mycobacterium abscessus infection presenting as a solitary pulmonary nodule in a 51yearold asymptomatic adult patient.