Giovanni Minisola

Cerebellar tumour presenting with pathological laughter and gelastic syncope

There is no report of patients in whom pathological laughter, a rare condition characterized by uncontrollable episodes of laughter usually triggered by unrelated stimuli, was ever closely associated with a loss of consciousness overtly linked with the onset of such uncontrollable laughter, also referred to as a gelastic syncope. A 53‐year‐old man presented with a 4‐month history of syncope following intense and uncoordinated laughter. Physical and neurological examination was normal and the patient had no other typical cerebellar signs. We found a mass in the cerebellar vermis abutting the floor of the fourth ventricle, which upon histological examination after surgery proved to be an ependymoma. We emphasize that pathological laughter and gelastic syncope could represent unique and sole features of a cerebellar disorder.

Probiotic lactobacilli: A new perspective for the treatment of inflammatory bowel disease

Inflammatory bowel disease, such as ulcerative colitis and Crohns disease, results from an interaction between susceptibility genes, the hosts bacterial environment, gut barrier defects, and immunological factors. New management approaches have been evolved from advances in our understanding of the pathobiology of this common gut disorder In particular, the therapeutic manipulation of the bacterial microenvironment in the gut seems to offer an innovative tool for the treatment of those patients. Since the gut is a highly sensitizing organ that contributes to the systemic immune response, potent treatments need to be developed to reduce gut inflammation in this disorder. Recent studies have demonstrated that probiotic lactobacilli, and also immunostimulatory DNA sequences from those same bacteria have an important anti-inflammatory potential in this context. Future research should better define among patients with inflammatory bowel disease the various clinical phenotypes with the greatest potential of response to probiotic treatment. Identification of the genes leading to the disease and a rather better understanding of the underlying immunoregulatory abnormalities will be crucial steps to define the different profiles of interaction between endogenous digestive bacterial flora and the immune system in each individual patient. Such advances will probably lead to targeting of effective treatments, including bacteriotherapy with probiotic lactobacilli, to subsets of patients with inflammatory bowel disease.

A patient with cerebral Whipple's disease and a stroke-like syndrome

The central nervous system (CNS) may be affected in up to 50% of patients with Whipples disease and this can occur even with little or no gastrointestinal involvement. We describe a 63-year-old patient in whom CNS involvement with Whipples disease had the clinical and imaging features of a brain infarction. Treatment with aspirin and ceftriaxone followed by trimethoprim-sulfamethoxazole resulted in a good neurological recovery and complete remission of the malabsorption syndrome. Cerebral Whipples disease resembling a stroke syndrome has so far been reported in only two other patients and in both cases it represented the first presentation of the disease. Arterial or arteriolar fibrosis, thrombosis and thickening associated with the inflammation of adjacent brain parenchyma and leptomeninges, and cerebral vasculitis caused by the hematogenous spread of Tropheryma whippelii to the brain may all be important triggers of brain infarction in patients with Whipples disease. Our case report highlights the important point that cerebral Whipples disease with the features of a stroke syndrome, if recognized early and treated aggressively with antibiotics, could have a favorable course with no long-term disability sequelae.

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME IN A PATIENT WITH BEHÇET'S DISEASE

We report on a patient who had a life-threatening relapse of Behc¸ets disease associated with a catastrophic antiphospholipid syndrome. The patient experienced over a short time a recurrent acute myocardial infarction, multiple venous thromboses, uveitis, and erythema nodosum. Search for thrombophilic factors was positive only for lupus anticoagulant (LAC) and criteria for the diagnosis of the antiphospholipid antibody syndrome were fulfilled. LAC was not found three months after the discharge. At that time the patient had no evidence of clinically active disease or thrombosis. We suggest that LAC was the main triggering factor for the repeated thromboses in this patient.

Rupture of the spleen after colonoscopy: a life-threatening complication.

During colonoscopy, the risk of injuring the spleen or other viscera except the colon is negligible. We report here a patient in whom spleen rupture did complicate the very early course of colonoscopy, but this remains an extremely rare complication with no more than 50 cases so far described. Diagnosis may be difficult, and the risk of spleen rupture seems to be greatest within 24 hours of colonoscopy. Mechanisms leading to spleen injury in the setting of colonoscopy are unclear; however, direct trauma, colon distension by insufflated air, and the excessive traction on the splenocolic ligament may be involved. Patients with splenomegaly and those with preexisting adhesions are at greater risk for this complication. Patients complaining of persistent abdominal pain after colonoscopy should be closely monitored and aggressively investigated for the suspect of spleen injury and rupture.

Pancreatitis during treatment with liraglutide.

A 67-year-old man presented with a 10-day history of nausea, vomiting, and constant pain in the epigastrium which radiated to the sides. Five months before admission, liraglutide (1.2 mg/day) had been started in addition to metformin and gliclazide for the treatment of type II diabetes and the dose had been stable. His previous history was otherwise unremarkable; he did not report any acute or chronic pancreatic disease and denied alcohol use, toxic habits or taking any other medications, including over-the-counter medications or herbal remedies, potentially associated with pancreatitis. The patient was fully alert and oriented, apyrexial and had normal vital signs; physical examination yielded normal findings apart from a severely tender abdomen with no bowel sounds and no rebound tenderness. Laboratory data showed increased blood amylase (877 IU/L; reference range: 0-115 IU/L), lipase (653 IU/L; reference range: 0-190 IU/L), alanine aminotransferase (ALT) (275 IU/L; reference range 10-36 U/L), aspartate aminotransferase (AST) 326 IU/L (normal range 10-36 IU/L), and total bilirubin 2.6 mg/dL (normal range 0.2-1.0 mg/dL) with conjugated bilirubin 0.8 mg/dL; γ-glutamyltransferase, alkaline phosphatase, electrolytes, hematological variables, cholesterol, triglycerides, renal function tests, and blood gases were normal. The results of serologic tests for the Mycoplasma and Chlamydia species, viral hepatitis and a wide range of other viral infections, and an autoimmunity screening were also negative. Findings of magnetic resonance imaging showed a moderately enlarged and edematous pancreas and sludge in the gallbladder but no stones neither dilatation of intra-and extra-hepatic biliary ducts, nor common bile duct and Wirsungs duct. Liraglutide was discontinued and the patient was managed conservatively with bowel rest and intravenous fluids; five days after admission, enzymes returned to normal and he was free of symptoms. A re-challenge test was not performed for safety reasons. We suspected, on clinical grounds, this patient had pancreatitis caused by exposure to liraglutide. This was lent support by scoring on the Naranjo probability scale that suggested a possible liraglutide-related event [1]. However, the finding of biliary sludge in the gallbladder is a confounding factor even though it was not associated with stones or sludge in, or dilatation of, the intra-and extra-hepatic biliary tract and Wirsungs duct on imaging studies. On a practical level, this substantially lessens the hypothesis of a biliary cause of pancreatitis in this patient. Furthermore, he had no prior history of acute or chronic pancreatic disorders and alternative causes of pancreatitis such as alcohol use, hypertriglyceridemia, hypercalcemia, autoim-munity, or …

Mirtazapine-Associated Hyponatremia in an Elderly Patient

Indications for ESA use included: CKD (57),off-label (30), myelosuppressivechemotherapy(7), and allogenicblood transfusionreduction (4). The CPOE rule fired in 3.7% (18/490) of ESA orders during the analysis.It stopped ESA prescribing94% (17/18)of the time. For the one alertoverride,the indication was CKD, hemoglobin level was 12.6 g/dL,and treatment plan requirement wasselected by theprescriber. Adverseeffects fromESAadministration werenotnotedfor thispatient. Discussion. Wehavevalidated theeffectiveness of therulewecreated by demonstrating a high percentage(>94%)of ESA orders prevented. Importantly, theoverride functionality was utilized in onlyone caseduring the analysis. Limitations includethe studysretrospective designand shorttimeperiod. A more longitudinal analysis to discernruleeffectivenessis needed.

Tiny Bubbles: Coronary Aneurysms and Lupus

PRESENTATION Among the cardiac complications that can be spurred by systemic lupus erythematosus, coronary aneurysms are a particularly challenging presentation. A 32-year-old woman with longstanding systemic lupus erythematosus was referred to us for treatment of New York Heart Association class III heart failure, including possible mitral valve replacement. She had diffuse proliferative lupus nephritis (class IV), chronic renal failure, hypertension, chronic anemia, and lung tuberculosis. She had no personal or family history of Kawasaki’s disease, rheumatic heart disease, or coronary artery disease. Her drug regimen consisted of ramipril, bisoprolol, prednisone, isoniazid, rifampicin and pyrazinamide. She did not complain of recent chest, back, or upper limb pain.

Probable Celecoxib-Induced Hepatorenal Syndrome

patientsare not receivingthe pneumococcal vaccinebecausethey were unaware it is neededor theirphysician had not recommended it to them. Discussing withpatients the indications for the pneumococcal vaccinein diabetes mellitus, as well as the revaccinationschedule, may increase pneumococcal uptake.Lastly,reachingout to local physiciansto aid in spreading awareness of the needfor thesevaccinations, especially pneumococcal vaccines, in diabeticpatientsmay help to furtherincreaseuptake.

Mesenteric Vein Thrombosis

To the Editor: In the November 2007 issue of Annals of Emergency Medicine, Hugon and colleagues appropriately cite a wide range of hereditary and acquired hypercoagulable disorders as an inherent and often underdiagnosed cause of portal and mesenteric thrombosis; however, no mention of hyperhomocysteinemia is made. We have recently observed two patients with a severe thrombosis in the portal and mesenteric districts in whom elevated blood levels of homocysteine were found at the time thrombosis was diagnosed; one of them was also heterozygous for factor V Leiden mutation. Hyperhomocysteinemia is known to represent an independent predisposing factor to splanchnic venous thrombosis but almost all the patients so far described in whom portal or mesenteric thrombosis was linked with hyperhomocysteinemia also had at least one additional prothrombotic disorder. Despite these apparent discrepancies, blood homocysteine should be measured in our opinion in every patient in whom a diagnosis of splanchnic venous thrombosis is made since a subtle but clinically meaningful underlying hyperhomocysteinemia is probably more common in the general population than commonly thought. We should keep in mind, for example, that exposure to any folate or vitamin B6 antagonist, such as methotrexate, phenytoin, estrogens, tobacco, or theophylline, could significantly raise blood concentrations of homocysteine thus strongly increasing the risk of arterial and venous thrombotic events. Additionally but not less important, blood homocysteine may be easily reduced to within the normal range by simply giving folic acid and B vitamins. We know that myeloproliferative disorders are the most common cause of portal and mesenteric thrombosis and the Budd-Chiari syndrome, which is a very important point. This suggests that, when the reason of such a severe and lifethreatening event as a splanchnic venous thrombosis is not overtly apparent, the patient has a great probability of having a myeloproliferative disorder. Even though frequently suspected, a definite diagnosis of a chronic myeloproliferative disorder in this setting remains a hard task even for skilled physicians since clinical and hematological parameters, including bone marrow biopsy, may yield inconsistent and insufficient or misleading information. Recent studies, however, have suggested that the V617F mutation of the JAK2 gene could be used as a very reliable and non invasive molecular marker for recognizing an underlying myeloproliferative disorder in patients with otherwise unexplained splanchnic venous thrombosis. Available data in fact appear to indicate a prevalence of the mutation that approaches rates as high as 50% in this population of patients. The test is obviously expensive, timeconsuming and requires molecular expertise; however, it may ultimately result in a cost-effective first-line screening assay for the early diagnosis of myeloproliferative disorders, at least in those patients in whom no other cause of the Budd-Chiari syndrome or thrombosis in the portal or mesenteric districts is found. We propose that, in this era of technological and molecular medicine, emergency physicians should rethink the current standard of care for patients presenting with splanchnic venous thrombosis and consider adding the search for V617F mutation of JAK2 gene in the diagnostic workup.