Persistent Anemia in the Setting of a Rare Pancreatic Pseudoaneurysm.

Introduction. Pancreatic pseudoaneurysm is a rare but potentially fatal complication that can follow pancreatitis. While early detection is critical to preventing poor long-term outcomes, clinical features vary significantly. Most often, abdominal pain is the presenting complaint, but this can be complicated as classic symptoms of pancreatitis also present with abdominal pain. Herein, we present a patient with an acute on chronic gastrointestinal bleed that was finally attributed to a pancreatic pseudoaneurysm. Case Presentation. The patient was a 56-year-old male with a past medical history significant for epilepsy, alcohol abuse, and hypertension who presented as a transfer from an outside facility for a gastrointestinal bleed. Prior to presentation, the patient reported rectal bleeding over the prior 1.5 months but had not sought care until bleeding increased along with increased abdominal pain. The patient's hemoglobin was 6.3 at presentation of the outside facility and received a total of four units of packed red blood cells (PRBCs) prior to arrival. After arrival, persistent bleeding was noted, and an additional 2 units of PRBCs were transfused. A computed tomography angiography (CTA) of the abdomen was obtained to identify the source for embolization. This, however, revealed a 4 × 4 × 3.5 cm intrinsically dense or enhanced mass of the pancreatic head. Discussion. Pancreatic pseudoaneurysm is a rare but potentially fatal complication that can follow pancreatitis. In chronic pancreatitis patients who underwent imaging incidence is estimated to be up to 10%. Treatment is difficult, and coil embolization is often used, though this can lead to splenectomy due to splenic ischemia. Stent grafts can be used in the surrounding arteries to maintain the integrity of viscera but carry risk of stent-related thrombosis. Further research is needed on the optimal management of this potentially lethal complication of pancreatitis.

The unique phenomenology of sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag")

Sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag") is an adult-onset, progressive, debilitating movement disorder first described in Filipino males from Panay Island in 1975. XDP manifests predominantly as torsion dystonia, later combined with or sometimes replaced with parkinsonism. Within the Island of Panay, the preva-lence rate is highest in the province of Capiz, where 1:4000 men suffer from the disorder. There is a high degree of penetrance and generalization. While women often serve as carriers, XDP is not limited to men. An updated XDP Philippine registry (as of January 2010) has identified 505 cases, with 500 males and 5 females. While some report that females may carry a milder form of the disorder, in our experience, both sexes generally follow a similar progressive clinical course.

The unique phenomenology of sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag").

Sex-linked dystonia parkinsonism (XDP, DYT3, "Lubag") is an adult-onset, progressive, debilitating movement disorder first described in Filipino males from Panay Islands in 1975. XDP manifests predominantly as torsion dystonia, later combined with or sometimes replaced with parkinsonism. Within the Island of Panay, the prevalence rate is highest in the province of Capiz, where 1:4000 men suffer from the disorder. There is a high degree of penetrance and generalization. While women often serve as carriers, XDP is not limited to men. An updated XDP Philippine registry (as of January 2010) has identified 505 cases, with 500 males and 5 females. While some report that females may carry a milder form of the disorder, in our experience, both sexes generally follow a similar progressive clinical course.

The natural history of sex-linked recessive dystonia parkinsonism of Panay, Philippines (XDP).

Sex-linked dystonia parkinsonism (XDP) was reported by Lee et al. in 1975 occurring endemically in Panay, Philippines. It is an adult onset, sex-linked, predominantly male, severe, progressive movement disorder with high penetrance and a high frequency of generalization. The movement disorder is characterized by dystonic movements usually starting in the third or fourth decade, focal at the onset, spreading to generalization within 2-5 years. The dystonia co-exist or is replaced by parkinsonism usually beyond the 10th year of illness. As of June 2001, 376 XDP cases have been registered. One hundred and fifteen cases have died. The prevalence of XDP in the island of Panay is 5.24 per 100,000; 0.34/100,000 in the general population. The prevalence varies in the different provinces; it is highest in Capiz at 18.88/100,000, 7.46/100,000 in Aklan, 1.28 in Iloilo and 0.83 in Antique. The 376 cases are from 188 families and 92% of cases have positive family history. Ninety-nine percent of the cases are males. The mean age of onset is 39.48 years. Duration of illness is 12.95 years. Ninety-four percent of patients initially manifest with dystonic symptoms, while only 6% present with Parkinsonian traits. Among those presenting with dystonia, the initial presentation is in the lower extremities in 33%, craniofacial in 27%, cervical and shoulder in 25%, upper extremities in 14%, and trunk in 1%. Regardless of the site of onset, the dystonia spreads in 98% and generalizes within 5 years in 84%. Neuroimaging (magnetic resonance imaging, MRI) was done in 16 patients. In the patients who have just manifested the disease usually when dystonia predominates and parkinsonism is absent. MRI showed minimal atrophy of the caudate and putamen or subtle putaminal signal abnormality. In the late course, where Parkinsonism predominates, severe atrophy of the caudate and putamen as well as marked increase in signal abnormality are seen. There are six autopsied cases of XDP. Neuropathology revealed marked atrophy of the caudate and putamen mostly in the cases with longstanding illness. The sex-linked pattern of inheritance has been established. Genetic studies have located the affected gene (DYT3) to Xq13.1. Nemeth's group has mapped the XDP gene to a <350 kb locus in the DXS 7117-DX 559 region. To date, no treatment has been proven consistently effective.