Oral Litholysis in Patients with Chronic Calcific Pancreatitis Unresponsive to or Ineligible for Extracorporeal Shock Wave Lithotripsy and Endoscopic Therapy.

BACKGROUND/AIMS: Our study aimed to evaluate the effect of oral litholysis in patients with chronic calcific pancreatitis (CCP) unresponsive to or ineligible for extracorporeal shock wave lithotripsy (ESWL) and endoscopic therapy. METHODS: Trimethadione, an antiepileptic agent, was administered orally at a dose of 0.6-0.9 g/day to 15 patients with this condition. Treatment outcome was evaluated by assessment of dissolution of the pancreatic stones on plain X-ray films and computed tomography scans of the upper abdomen. Plasma glucose, hemoglobin A1c, and body mass index (BMI) were also monitored throughout the therapy. RESULTS: Litholysis was observed in 13 out of 15 patients (86.7%) and was definite in 10 and partial in 3. Six patients had pancreatitis attacks during the therapy; 5 of whom showed definite litholysis and had only 1 mild attack. Of the 11 patients with normal or impaired glucose tolerance at baseline, none developed diabetes mellitus and all showed litholysis. BMI significantly increased in patients whose pancreatic stones dissolved. There was no vital organ impairment by trimethadione. CONCLUSION: Oral litholysis using trimethadione may represent a noninvasive and effective complementary treatment in patients with CCP unresponsive to or ineligible for ESWL and endoscopic therapy.

Effectiveness of oral litholysis therapy for improving glucose intolerance and malnutrition in patients with poor results following endoscopic therapy and extracorporeal shock wave lithotripsy for calcified pancreatic stones.

We report a case of pancreatolithiasis in which glucose intolerance and malnutrition were significantly improved after starting oral litholysis therapy (OLT) with use of trimethadione. A 43-year-old female with multiple calcified stones in the main and peripheral pancreatic ducts had experienced recurrent and severe attacks of pain for 7 years (from 21 to28 years of age). Impaired glucose tolerance was first noted at the age of 32 years. We started OLT after interventional endoscopic therapy combined with extracorporeal shock wave lithotripsy failed because of kink and stenosis of the main pancreatic duct (MPD). Over the next 9 years, a significant decrease in total pancreatic calcified stone volume was shown by computer analysis of follow-up computed tomography images. Larger stones completely disappeared without attacks of pain. In addition, both glucose intolerance and insulin secretion were significantly ameliorated, followed by improvement of malnutrition. OLT may induce intraductal decompression by dissolving stones in the peripheral ducts as well as the MPD, with resulting preservation of endocrine function and improvement of malnutrition. Since the present results were obtained in a single case, further clinical trials are necessary to evaluate the value of performing OLT under various conditions to eliminate stones.

Respiratory failure in a patient with antecedent poliomyelitis: amyotrophic lateral sclerosis or post-polio syndrome?

We report a 69-year-old man who developed paralytic poliomyelitis in childhood and then decades later suffered from fatal respiratory failure. Six months before this event, he had progressive weight loss and shortness of breath. He had severe muscular atrophy of the entire right leg as a sequela of the paralytic poliomyelitis. He showed mild weakness of the facial muscle and tongue, dysarthria, and severe muscle atrophy from the neck to proximal upper extremities and trunk, but no obvious pyramidal signs. Electromyogram revealed neurogenic changes in the right leg, and in the paraspinal, sternocleidomastoid, and lingual muscles. There was a slight increase in central motor conduction time from the motor cortex to the lumbar anterior horn. Pulmonary function showed restrictive ventilation dysfunction, which was the eventual cause of death. Some neuropathological features were suggestive of amyotrophic lateral sclerosis (ALS), namely Bunina bodies. In patients with a history of paralytic poliomyelitis who present after a long stable period with advanced fatal respiratory failure, one may consider not only respiratory impairment from post-polio syndrome but also the onset of ALS.

Multiple cerebellar infarcts: clinical and pathophysiologic features.

We investigated the clinical features of 15 patients with multiple isolated ischemic lesions in the cerebellum. The main initial symptoms were vertigo and nausea/vomiting in 8 patients each, and headache in 6 patients. Dysarthria and cerebellar ataxia of the limbs and trunk were observed in 14 patients. The ischemic lesions were in the posterior inferior cerebellar artery (PICA) plus superior cerebellar artery (SCA) territories in 4 patients (27%), and PICA plus SCA plus anterior inferior cerebellar artery (AICA) territories in 3 patients (20%). Infarcts were in the AICA plus SCA territories in 2 patients (13%), PICA only in 2 patients (13%), and SCA only in 4 patients (27%). Hemorrhagic lesions were observed in the ischemic area in 2 patients (13%). Vascular lesions included occlusive lesions of the vertebral arteries in 9 patients (60%), but no abnormalities of the vertebrobasilar arterial system were observed in 6 patients (40%). The functional prognosis was good in most cases. The underlying mechanism was atherothrombotic, including arteriogenic embolism in 9 patients (60%) and cardiogenic embolism in 3 patients (20%). An embolic mechanism was considered in all cases, but in some cases an accurate pathogenesis of the cerebellar infarction was obscure.

Rapidly progressive fatal respiratory failure (Ondine's curse) in the lateral medullary syndrome.

A 70-year-old man presented with unilateral lateral medullary infarction, and then died of rapidly progressive respiratory failure within a day. The clinical manifestations were hiccups, hoarseness, dysarthria, nystagmus, left central facial paralysis, paralysis of the left soft palate, dysphagia, decreased superficial sensation over the right face and upper limb, and cerebellar ataxia in the left upper and lower limbs. The arterial blood gas analysis revealed mild hypoventilation. Soon thereafter, an apneic episode occurred during a sleep and advanced to ataxic respiration, and the patient died. Pathologically, there were fresh ischemic infarction localized to the left dorsolateral area of the upper medulla, caused by atherothrombotic occlusion of the left vertebral artery. These foci were in the areas including the medullary reticular formation, the solitary nucleus, the intramedullary fibers of the vagus nerve, and the nucleus ambiguus on the left side. We attributed the fatal acute progressive respiratory impairment in the present case to impairment of the automatic respiratory system (Ondine's curse) rather than the voluntary respiratory system.

[Analytical studies of both initial symptoms and clinical symptoms and signs of different etiologies of chronic pancreatitis: an approach by using odds ratios].

We conducted the statistical analysis of both initial symptoms and clinical symptoms and signs of different etiologies of chronic pancreatitis by using odds ratios which was one of the techniques of evidence-based medicine. The official report published by The Research Group of Intractable Pancreatic Diseases sponsored by the Welfare Ministry of Japan in 1986 was available as the data source of the present study. Nine items of initial symptoms and 25 items of 28 clinical symptoms and signs were compared in 4 different etiologies of the disease which were alcoholic, biliary, idiopatic and nonalcoholic (both biliary and idiopatic). In initial symptoms, 1.5 items were significantly more observed in alcoholic pancreatitis than in nonalcoholic, biliary and idioatic pancreatitis, 4 of which (abdominal pain, back pain, poor appetite and loss of body weight) were common items as might be related closely to the alcohol intake, 2. only one item of jaundice was significantly more observed in biliary pancreatitis than in alcoholic and idiopatic pancreatitis, 3.3 items of poor appetite, diarrhea and abdominal mass were more frequently observed in idiopatic pancreatitis than in biliary pancreatitis. In clinical symptoms and signs, 1. almost all items (21 to 24) were significantly more observed in alcoholic pancreatitis than in the other etiologies of the disease, and seemed to be related directly or indirectly to alcohol intake, 2.3 or 4 items which were related closely to gallstone and acute cholecystitis were significantly more observed in biliary pancreatitis than the other two etiologies of the disease, and 3.4 items consisting of diarrhea, loss of body weight, and pancreatic swelling were more frequently observed in idiopathic pancreatitis than in biliary pancreatitis.