Reversible Cerebral Vasoconstriction Syndrome and Ischemic Stroke Secondary to Peripartum Cardiomyopathy - Report a Rare Case.

Acute cardioembolic stroke is a rare presentation of peripartum cardiomyopathy. We present an unusual case of peripartum cardiomyopathy, that subsequently developed cardioembolic ischemic stroke and reversible cerebral vasospasms.  A 26-year-old G1P1 caucasian woman presented to the emergency department 10 days after a spontaneous vaginal delivery with the clinical and physical presentation of acute heart failure. Brain natriuretic peptide (BNP) level was >8000 pg/mL. Transthoracic echocardiogram (TTE) demonstrated global left ventricular hypokinesis, reduced ejection fraction (EF) 22% with grade I diastolic dysfunction and apical thrombus. On hospital day two of her heart failure exacerbation admission, a code stroke was activated for aphasia and confusion. She received an IV tissue plasminogen activator (tPA) and underwent a mechanical thrombectomy. On hospital day three, she developed worsening of neurological symptoms, and a computed tomography (CT) angiogram revealed vasospasm in the region of the left middle cerebral artery (MCA), which subsequently resulted in nimodipine therapy. Furthermore, her hospital course was complicated by persistent hypotension, and with our concern for vasospasm that was noted in the CT angiogram instead of guideline-directed therapy for heart failure, digoxin was given to control heart rate and to improve cardiac output. Ultimately, her neurological symptoms improved, and she was discharged on hospital day 10. This case highlights the combination of rare presentations - postpartum cardiomyopathy, ischemic stroke, and reversible cerebral vasospasms, which suggests that the time and size of the stroke are of the essence in terms of promptness of aggressive treatment.

Not Lupus Nephritis but a Rare Case of Drug-Induced Pauci-Immune Glomerulonephritis.

Hydralazine-induced pauci-immune glomerulonephritis is a rare cause of glomerulonephritis. It is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis that can be rapidly progressive and potentially life-threatening. However, most cases are found to be asymptomatic, and patients often present with acute renal failure and painless hematuria. It has been confused with lupus nephritis but treatment differs, thus, necessitating the need for differentiation. A case report of an 80-year-old African American woman with a history of hypertension, diabetes mellitus type 2, and hypothyroidism, who presented with generalized weakness and weight loss of 30-40 lbs. The patient had been treated with hydralazine for months for hypertension. She presented to the hospital with acute renal failure that worsened over the course of several months eventually requiring hemodialysis. The patient was found to have drug-induced ANCA vasculitis from hydralazine. This etiology was confirmed with pauci-immune glomerulonephritis seen on renal biopsy. This presentation has the potential of being confused with lupus nephritis. Despite the initial serology being suggestive of lupus, this type of nephritis does not have positive immunofluorescence. The treatment of nephritis in this patient was generally supportive. However, it was important to identify the underlying cause of renal failure. Equally important to initiating immunosuppressive therapy, it was imperative to discontinue the offending drug in a timely manner to prevent rapid organ failure. The causative agent, hydralazine, may have otherwise gone unnoticed without a thorough investigation into other causes of renal failure. Thus, it is important to consider this as a diagnosis with a patient who presents with rapidly progressive renal failure on hydralazine and may mimic lupus nephritis.

A Rare Case of Spontaneous Tumor Lysis Syndrome in Hodgkin Lymphoma.

Tumor lysis syndrome (TLS) is an oncological emergency characterized by biochemical abnormalities such as metabolic acidosis, hyperkalemia, hyperphosphatemia, and hypocalcemia. The clinical outcome is directly related to the biochemical abnormalities. TLS can occur in any malignancy, but it is highly associated with rapidly proliferating tumors. Although the syndrome is commonly associated with hematological malignancies, particularly with leukemia and non-Hodgkin's lymphoma, it is rarely seen in patients with Hodgkin's lymphoma. In our case, a 7-year-old girl presented with intermittent fever, non-productive cough, fatigue, and night sweats for four months. On examination, she had an enlarged cervical lymph node of 5 cm in size on the left side accompanied by palpable supraclavicular lymphadenopathy. Past medical history was significant for the relapsing and remitting course of nephrotic syndrome diagnosed two years before presentation. The patient underwent a left-sided cervical node excisional biopsy, which confirmed classical Hodgkin's lymphoma of mixed cellularity type. Her baseline chest x-ray revealed a bulky anterior mediastinal mass. To stage the tumor, a bone marrow biopsy, CT, and positron emission tomography (PET) scan were done. Although the bone marrow biopsy report showed a normal pattern of trilineage hematopoiesis, the CT and PET scan results led to its classification under stage 4. During her stay in the hospital for further work-up and treatment, her condition suddenly deteriorated. There were biochemical derangements on lab reports that confirmed the Spontaneous Tumor Lysis Syndrome (STLS). She recovered completely due to immediate stabilization and correction of electrolyte abnormalities. STLS is a life-threatening condition that is rarely seen in patients with Hodgkin's lymphoma. The treating physicians should be vigilant about this possible sequela of Hodgkin's lymphoma and be aware of its different presentations.

Lamin A/C Cardiomyopathy with E203K Pathogenic Mutation.

Lamin A/C (LMNA) cardiomyopathy is an adult-onset, autosomal dominant, rapidly progressive cardiomyopathy which belongs to a spectrum of familial idiopathic cardiomyopathies. It is the most common type of familial dilated cardiomyopathy that is associated with conduction defects. A 76-year-old African American female with second-degree atrioventricular (AV) block presented for evaluation of persistent fatigue. Her family history was significant for sudden deaths of her son and brother at the age of 6 and 48 years, respectively, and AV block in her sister with a pacemaker implant at the age of 64 years. Physical examination was within normal limits. Electrocardiogram showed a Mobitz type II, second-degree AV block. Mild dilated cardiomyopathy was present on echocardiogram. Stress echocardiography had to be stopped due to premature ventricular contractions. Cardiac catheterization, coronary angiography, and cardiac MRI revealed no significant etiology for rhythm disturbance. Holter monitoring revealed intermittent bradycardia with a heart rate falling as low as 28 beats per minute, which led to the decision of dual-chamber pacemaker implantation. RhythmNext genetic testing (Ambry Genetics, Aliso Viejo, CA) was done due to the significant family history of sudden death; it revealed a heterozygous E203K pathologic mutation in the LMNA gene. Sudden death is the most common mode of death in LMNA cardiomyopathy; hence, the implantation of intracardiac cardioverter-defibrillator for primary prophylaxis was discussed with the patient. Clinicians should suspect LMNA cardiomyopathy in patients with rhythm disorders and family history of sudden death, which can help to identify individuals at risk and prevent sudden death by appropriate interventions.