Proton Pump Inhibitors and Risk of Dementia: A Hypothesis Generated but Not Adequately Tested.

We reviewed the evidence on proton pump inhibitors (PPIs) and dementia. PPIs are among the most widely utilized drugs in the world. Dementia affects roughly 5% of the population of the United States (US) and world aged 60 years and older. With respect to PPIs and dementia, basic research has suggested plausible mechanisms but descriptive and analytic epidemiological studies are not inconsistent. In addition, a single large-scale randomized trial showed no association. When the evidence is incomplete, it is appropriate for clinicians and researchers to remain uncertain. Regulatory or public health authorities sometimes need to make real-world decisions based on real-world data. When the evidence is complete, then the most rational judgments for individual patients the health of the general public are possible At present, the evidence on PPIs and dementia suggests more reassurance than alarm. Further large-scale randomized evidence is necessary to do so.

Glycogen Hepatopathy: A Reversible yet Relapsing Cause of Hepatitis in Type 1 Diabetics.

Glycogen hepatopathy (GH), a rare glycogen storage disease caused by genetic or acquired overactivation of hepatic glycogen synthesis enzymes, can mimic non-alcoholic fatty liver disease (NAFLD). We describe a case of biopsy-proven GH in an adult with type 1 diabetes mellitus (DM). A 33-year-old Honduran woman with a 25-year history of type 1 DM complicated by gastroparesis, multiple episodes of diabetic ketoacidosis (DKA) and hypoglycemia, and recurrent pancreatitis was referred for abnormal liver enzymes. Family history was negative for liver disease. There was no history of alcohol or recreational drug use. Patients' medications included insulin and thyroxine. Physical exam showed hepatomegaly but no stigmata of chronic liver disease. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) had ranged from 100's to over 7000 U/L while alkaline phosphatase (ALP) was elevated to over 400 IU/L. Albumin, total bilirubin, platelets, international normalized ratio (INR), eosinophils, viral hepatitis panel, antinuclear antibody (ANA), smooth muscle antibody (Ab), anti-liver-kidney microsomal (LKM) Ab, celiac serologies, ceruloplasmin, alpha 1 antitrypsin, iron studies, and acetaminophen levels were all normal. An abdominal ultrasound showed "fatty liver" and an atrophic pancreas. CT abdomen showed hepatomegaly. The common bile duct (CBD) was found to be normal on endoscopic ultrasound (EUS) and magnetic resonance cholangiopancreatography (MRCP). A liver biopsy was pursued eventually, demonstrating glycogenotic hepatocytes. GH is frequently misdiagnosed as NAFLD, a more common liver disease that occurs in association with diabetes While GH is known to be reversible, NAFLD has been known to progress to advanced liver disease, ranging from cirrhosis to hepatocellular carcinoma. Definite diagnosis often requires liver biopsy because of overlapping clinical and radiographical pictures. Elevation of both glucose and insulin levels in the setting of fragile DM control is thought to play a role via overstimulation of glycogen synthesis. Recommended treatment is stable "tight" glycemic control; pancreatic transplantation has resulted in sustained GH remission in two case reports. The required degree of stability and tightness of glucose control is not yet known. An increased awareness of GH is needed in an attempt to prevent delay in diagnosis, in a condition with an otherwise unknown incidence.

Ileal Signet Ring Cell Carcinoma Masked by Crohn Disease.

Background: Signet ring cell carcinoma (SRCC) is a rare, highly malignant adenocarcinoma that generally involves the stomach; ileal involvement is uncommon. Crohn disease (CD) is associated with long-standing inflammation that may predispose to small intestine adenocarcinoma. Case Report: A 67-year-old male with ileal CD since age 23 years, maintained in remission by mesalamine, presented with mild intermittent attacks of abdominal cramping, an increase in bowel movements from 3 to 5 daily, and bloating for 3 months. Computed tomography enterography with contrast enhancement demonstrated 2 segments of ileal wall thickening. Colonoscopy performed 7 years prior was unremarkable. The patient received oral prednisone with mild symptomatic improvement; he declined biologics. Ileocolonoscopy 1 month later revealed a nontraversable terminal ileal stricture 15 cm from the ileocecal valve. Biopsy demonstrated signet ring cells infiltrating the lamina propria. The patient underwent laparoscopic ileocecectomy and ileocolic anastomosis. Histopathology of a 2.5-cm ileal mass showed poorly differentiated adenocarcinoma with mucin production and signet ring cell features. One metastatic mesenteric lymph node was identified. Adjuvant chemotherapy was initiated. Conclusion: This case of metastatic ileal SRCC occurred in the setting of long-standing, clinically controlled CD. Although the absolute risk of small-bowel adenocarcinoma in CD is low, active surveillance for small-bowel adenocarcinoma in patients with longstanding CD may be prudent, given the overlapping symptomology of SRCC and CD, the aggressiveness of SRCC, and the association of SRCC with subclinical inflammation.

Ziprasidone-Induced Oculogyric Crisis in a 74-Year-Old Female.

Oculogyric crisis is a rare ocular dystonia first appearing at the turn of the last century in postencephalitic patients. In the modern era, they were most frequently associated with first-generation D1 dopaminergic receptor blocking antipsychotic medication. We present an unusual case of acute oculogyric crisis in a 74-year-old woman with long-standing Parkinson disease following exposure to the second-generation neuroleptic ziprasidone, which has dopaminergic (D2) and serotoninergic (5-HT2A) receptor blocking effects and is used for severe delusions and psychosis. To the best of our knowledge, there are no other published reports.

Case Report: An Incidental Finding of Fahr's Disease in a Patient with Hypochondria.

Idiopathic basal ganglia calcification (IBGC), commonly referred to as Fahr's disease, is a rare neurological disorder characterized by the abnormal, symmetrical, and bilateral calcification of the basal ganglia and other brain regions. Patients typically present in their forties and fifties with various neurologic and/or psychiatric symptoms, including movement disorders, Parkinsonism, psychosis, and depression. The pathophysiology of this disease is not completely understood; however, several gene mutations have been identified in the pathogenesis of Fahr's disease. These mutations display an autosomal dominant inheritance pattern. Furthermore, the regional phenotypic expression of calcifications differs greatly from patient to patient, as do their clinical presentations. Here, we describe a patient who presented with psychiatric manifestations and imaging consistent with Fahr's disease.