A Progressive Multifocal Neurological Syndrome in a 42‐year‐old Woman

Abstract

Hospital Physician July 2008 35 P ancreatic cholera syndrome, also known as Verner-Morrison syndrome 1 and WDHA syndrome , is a constellation of symptoms caused by VIPomas, a rare subtype of neuroendo-crine tumor (NET) that typically originates in the pancreas. In this syndrome, excessive production of vasoactive intestinal polypeptide (VIP) provokes secre-tory diarrhea with associated electrolyte imbalance. VIPomas account for less than 1% of all pancreatic tumors and are usually solitary lesions larger than 3 cm, with 75% occurring in the tail of the pancreas. More than 60% of VIPomas metastasize by the time of diagnosis. 2 Diagnosis is dependent on confirming the presence of hormone hypersecretion and localizing the tumor using available imaging modalities. This article presents the case of a woman with the classic presentation of VIPoma who required multiple hospitalizations due to life-threatening hypokalemia. The approach to diagnosis of VIPoma and management of components of pancreatic cholera syndrome are also discussed. CASE PRESENTATION History and Physical Examination A 42-year-old woman with no significant past medical history presented to her primary care physician with abdominal pain 15 days after an elective tubal ligation. Initially, the symptoms were thought to be related to the postoperative course and the patient was treated with opiates. However, symptoms persisted for 2 months and were associated with bouts of copious, watery, non-bloody diarrhea (occurring 3-4 times/day). She denied recent use of antibiotics or laxatives. Current medications were acetaminophen/oxycodone to control pain. She had no known drug allergies and denied a history of smoking or illicit drug use. Previous surgeries included the recent laparoscopic tubal ligation, cholecystectomy, breast augmentation, and tonsillectomy. Family history was noncontributory. The primary care physician ordered esophagogastroduodenoscopy and colonoscopy, both of which were unremarkable. Abdominal computed tomography (CT) performed 6 months prior as part of the preoperative evaluation for tubal ligation was also normal. The patient's symptoms worsened, with 8 to 10 bowel movements per day, generalized fatigue, and a 26-lb weight loss, leading to an emergency department (ED) visit. In the ED, the patient's vital signs included a temperature of 99.8°F, blood pressure of 109/60 mm Hg, a regular heart rate of 98 bpm, respiratory rate of 20 breaths/min, and oxygen saturation of 99% on room air. Physical examination revealed abdominal distension, increased bowel sounds with no palpable masses or or-ganomegaly, and a normal rectal examination. Diagnostic Studies Laboratory values on admission were notable for elevated alkaline phosphatase (348 U/L), aspartate aminotransferase (83 U/L), and alanine aminotrans-ferase (49 U/L) levels. The potassium level was low at 2.4 mEq/L (normal, 3.5-5.0 mEq/L) and remained low despite daily oral potassium replacement. Stool cultures and samples for ovae, parasites, and Clostridi-um difficile toxin were negative....

Intravascular large B-cell lymphoma presenting as rapidly progressive dementia and stroke

“…[ 2 ] IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time. [ 3 ] Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes. [ 4 ] The diagnosis is challenging and frequently made at autopsy or biopsy.…”

“…IVLBCL displays a relatively high frequency of central nervous system (CNS), skin, and bone marrow involvement [2] . IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time [3] . Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes [4] .…”

Intravascular large B-cell lymphoma presenting as rapidly progressive dementia and stroke

“…[ 2 ] IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time. [ 3 ] Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes. [ 4 ] The diagnosis is challenging and frequently made at autopsy or biopsy.…”

“…IVLBCL displays a relatively high frequency of central nervous system (CNS), skin, and bone marrow involvement [2] . IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time [3] . Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes [4] .…”

Intravascular large B-cell lymphoma presenting as rapidly progressive dementia and stroke

“…[ 2 ] IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time. [ 3 ] Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes. [ 4 ] The diagnosis is challenging and frequently made at autopsy or biopsy.…”

“…IVLBCL displays a relatively high frequency of central nervous system (CNS), skin, and bone marrow involvement [2] . IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time [3] . Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes [4] .…”

Intravascular large B-cell lymphoma presenting as rapidly progressive dementia and stroke

“…[ 2 ] IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time. [ 3 ] Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes. [ 4 ] The diagnosis is challenging and frequently made at autopsy or biopsy.…”

“…IVLBCL displays a relatively high frequency of central nervous system (CNS), skin, and bone marrow involvement [2] . IVLBCL limited to the CNS is infrequent, and absence of extraneural features may result in delaying diagnosis for quite some time [3] . Neurologic symptoms were highly heterogeneous, including dementia, hemiparesis, seizures, myoclonus, and mental changes [4] .…”