Hyperglycemic Hemichorea: A Case Report.

Chorea is a hyperkinetic movement disorder characterized by a continuous flow of rapid, random, and involuntary bodily contractions, usually affecting the distal limbs. When these movements are more proximal or assume a larger amplitude with a flinging or kicking character, they're referred to as ballism. These disorders can be associated with several causes, ranging from genetic and neurovascular to toxic, autoimmune, and metabolic. Non-ketotic hyperglycemic hemichorea-hemiballismus is a rare consequence of decompensated diabetes mellitus with a poorly understood pathogenesis but with characteristic MRI T1 and T2 hyperintense abnormalities in the contralateral basal ganglia. We present the case of a 74-year-old woman with a history of poorly controlled type 2 diabetes mellitus, dyslipidemia, and arterial hypertension who was admitted to the emergency room due to a two-day history of rapid non-stereotypical involuntary movements of the left side of her body. A neurological exam showed large amplitude and repetitive left-side body movements. Glycemia was 541 mg/dL with no ketosis. Her glycosylated hemoglobin was 14%. A brain CT excluded acute abnormalities. Brain MRI showed a discrete T1 hyperintense signal involving the right corpus striatum, compatible with non-ketotic hyperglycemic hemichorea-hemiballism syndrome. After metabolic optimization with insulin and haloperidol, the movements resolved. Early recognition and metabolic control are essential to the resolution of choreiform movements. Our aim is to raise awareness for hyperglycemic hemichorea-hemiballismus, in which decompensated diabetes is the early sign of diagnosis.

Hypoglycemia in a Non-diabetic Patient and the Side Effects of Diazoxide Use.

A low blood glucose level (less than 55 mg/dL) associated with autonomic and neuroglycopenic signs and symptoms that resolve after glucose administration establishes Whipple's triad, indicating the presence of a hypoglycemic disorder. Insulinoma remains the most common cause of endogenous hyperinsulinemia. We present the case of a 73-year-old male who was brought to the emergency department after losing consciousness. On initial assessment, severe hypoglycemia was identified and treated. No abnormalities were detected on the physical examination, initial blood tests, abdominal ultrasound and computed tomography (CT) thorax, and abdomen and pelvis. The patient had another episode of symptomatic hypoglycemia, and the blood tests performed were compatible with endogenous hyperinsulinism. The patient was started on diazoxide to prevent further hypoglycemia episodes. Magnetic resonance imaging (MRI) showed a nodular area in the cephalic region of the pancreas, and the patient was discharged with diazoxide and flash glucose monitoring. In the follow-up appointment, he presented with signs and symptoms of congestive heart failure. Endoscopic ultrasound was requested, but the patient was at high risk for complications while undergoing the procedure under anesthesia due to congestive heart failure. A 68Gallium-DOTA-NOC positron emission tomography and computed tomography (PET-CT) was requested and confirmed the presence of a nodular area in the cephalic region of the pancreas. He was referred to general surgery for definitive treatment. Insulinoma is still a challenging medical condition. Therefore, management by a multidisciplinary team is essential. This case highlights the impact that side effects of medication used to treat this condition can have. Diazoxide was initiated to stop severe recurrent hypoglycemia; however, the patient developed congestive heart failure and was unable to undergo an endoscopic ultrasound to localize the lesion, resulting in a delay in diagnosis and definitive treatment. Diazoxide is a potent hyperglycemic drug but it can also cause fluid retention, nausea, hypertrichosis, neutropenia, and thrombocytopenia.

Leprosy in a Patient With Lymphoma: A Challenge in the Twenty-First Century.

Leprosy, or Hansen's disease, mistakenly considered a disease from the past by some, is still common nowadays, especially in tropical and subtropical regions. In the absence of appropriate medical treatment, it may progress and cause permanent damage to multiple organs. This case report illustrates the diagnostic challenge of a south-american adult man who had been living in Europe for over 14 years. He was referred to the Hematology department due to persistent lymphocytosis and a CD5+ B-cell lymphoproliferative disorder was identified. During clinical surveillance, the patient developed skin lesions in his limbs with associated hypoesthesia. A histological diagnosis of lepromatous leprosy was made, and he underwent a long-term three-drug therapeutic regimen (dapsone, rifampicin, and clofazimine). Adding to the complexity of the case, the patient progressed with splenomegaly and constitutional symptoms, more than 7 years after development of lymphocytosis. Through a comprehensive evaluation, a definitive diagnosis of mantle cell lymphoma was established and received 6-cycle R-CHOP induction, followed by maintenance rituximab. Importantly, prophylaxis for leprosy reactivation was not administered as there were no recommendations in available guidelines. Eventually, the patient experienced a leprosy relapse while on maintenance therapy, 58 months after completing the initial anti-leprous treatment. Clinical response was attained with a new treatment regimen consisting of rifampicin, clofazimine, and minocycline.  Although leprosy is primarily observed in tropical and subtropical regions, the long incubation period of this disease combined with the global flow of migrants, made us consider it. Despite being rare, leprosy relapses can occur even after a few decades. The contribution of rituximab or previously administered chemotherapeutic agents is still unknown. The question remains whether antibiotic prophylaxis should be performed in patients undergoing immunochemotherapy for malignant diseases.

Pheochromocytoma: A Case Report.

Pheochromocytomas are rare tumors located in the adrenal medulla, that derives from the chromaffin cells and produce catecholamines. They are an uncommon cause of hypertension, and only 50% of the patients present symptoms compatible with this pathology. Here we describe the case of a 70-year-old woman with a history of anxiety, hypertension and palpitation, who had an unspecified nodule in the right adrenal gland. Laboratory studies revealed an elevated urinary metanephrines secretion. A diagnosis of pheochromocytoma was made and an adrenalectomy was performed. Our aim is to highlight the diagnosis of this rare tumor and how its early management can prevent morbidity and mortality.

Secondary Amyloidosis and Common Variable Immunodeficiency: A Rare Association.

Common variable immunodeficiency (CVID) is a disease characterized by severe antibody deficiency due to impaired B cell differentiation. It represents the most common form of primary immunodeficiency in children and adults, and its clinical manifestations include recurrent infections and chronic lung disease, gastrointestinal infections, and autoimmunity. Here, we present the case of a 47-year-old female patient with a history of CVID and recurrent Campylobacter jejuni bacteremia. She was undergoing biweekly administration of intravenous immunoglobulin for over 15 years. During hospitalization rapidly progressive oliguric renal failure was observed in association with anasarca and nephrotic syndrome. Bilateral nephromegaly was noted on an abdominal pelvic computed tomography scan. Renal biopsy was consistent with amyloidosis, and serum amyloid A protein was elevated. The diagnosis of AA amyloidosis secondary to CVID was made. The patient was started on hemodialysis and weekly intravenous immunoglobulin administration with favorable clinical outcomes.