Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis (LCH) that affects different body systems. It was recently recognized as a neoplastic disorder after identifying an activating mutation of the MAPK pathway. Neurological presentations of ECD are rare. We present a case of a 35-year-old male who presented to the emergency department with neck pain, headache and vomiting for 2 months; MRI showed multiple heterogeneous intracranial masses. Neurosurgery performed a suboccipital craniotomy, partially resected the cerebellar mass, and placed a parietal to frontal shunt catheter. Biopsy results from the cerebellar mass demonstrated cerebellar tissue involved by a diffuse proliferation of foamy histiocytes and spindle cells admixed with prominent lymphoplasmacytic infiltrate and positive for CD68, CD163, Factor XIIIa and Fascin. PET scan showed hypermetabolic uptake within the medullary portions of the diffuse abnormal lesions of the distal femurs, tibias, and fibulas, and cardiac MRI was nonsignificant. The patient was started on vemurafenib and continued to show improvement in a 3-month outpatient follow-up.

No abstract

While it is primarily thought of as a respiratory illness, COVID-19 is now recognized as a multi-organ disease that can present with a wide range of clinical manifestations. Particularly in patients with severe respiratory illness, neurological manifestations ranging from headaches, and loss of smell to strokes have been associated with the virus. In the setting of resolving respiratory illness, it is important to consider autoimmune encephalitis (AE) in the instance of new-onset neurological manifestations. The typical patient presentation includes altered mental status, fever, seizures, and/or focal neurological deficits. These neurological manifestations make it crucial to consider either underlying COVID-19 infection or post COVID-19 autoimmunity so as not to delay the administration of the appropriate treatment. Herein, we present the case of an 88-year-old female with new-onset right leg weakness, and dysarthria, that progressively developed to altered mental status months after having respiratory symptoms of COVID-19. According to the criteria of AE diagnosis, the patient's clinical course and work-up findings proved the diagnosis.

Colpocephaly, a congenital anomaly in the ventricles, is usually discovered early in infancy and rarely during adulthood. Partial or complete agenesis of the corpus callosum or Chiari malformations (developmental arrest of white matter formation in early fetal development) can lead to abnormal asymmetrical ventricular enlargement. Minimal literature about colpocephaly is available for clinicians, making diagnosis and treatment very challenging. Colpocephaly in adults is an infrequent condition, mostly found as an incidental finding with no neurological or cognitive impairment. Symptoms usually represent the affected lobe in the brain as our patient's visual hallucination may be attributed to the lesioned occipital horn. Differentiating from normal pressure hydrocephalus, representing new-onset dementia, can help avoid unnecessary procedures. Neurological and psychiatric consultation should be practiced to exclude other causes of neurological and cognitive impairment. While there is no definitive treatment for this condition, seizure prophylaxis has been helpful. Cognitive behavioral therapy, psychotherapy, and social skills training are recommended in some literature. Typical and atypical antipsychotics can control symptoms with uncertain efficacy.

Human herpesvirus-6 (HHV-6) is a virus known for causing the highly contagious infection, roseola infantum, and has been associated with causing encephalitis in pediatric patients and less commonly in adult patients as well. Regardless of the patient's age, the primary HHV-6 infection could be complicated by neurological sequelae including encephalitis, acute encephalopathy with biphasic seizures syndrome, or demyelinating disease. HHV-6 encephalitis does occur in an adult as a primary infection or reactivation. However, immunocompromised, hematopoietic stem cell transplantation patients, and solid organ transplant recipients are the most affected population. Here we present a rare case of HHV-6 encephalitis in a 26-year-old healthy immunocompetent male. HHV-6 viral DNA was detected in the cerebrospinal fluid during the acute stage of the disease, and the diagnosis was confirmed by quantitative polymerase chain reaction (PCR). The patient was treated with ganciclovir and had a complete response to treatment without any further complication. The pathophysiology, clinical course, and treatment in otherwise immunocompetent adult patients are also discussed.

No abstract

Rosai–Dorfman disease (RDD) is a rare disorder characterized by the proliferation of histiocytes in lymph nodes. It can occur anywhere in the body but commonly involves the cervical area of the neck. Its clinical presentation varies and patients with skin manifestations may develop papules, nodules, plaques, or pustules. Histologically, it typically presents with emperipolesis, where intact lymphocytes are found within histiocytes. The definitive treatment of RDD is not well established given the rarity of the disease and indeed skin lesions can regress spontaneously. Therapeutic treatment options include cryotherapy, radiation, or topical agents such as steroids or retinoids. Here we describe the case of a 24-year-old Hispanic female who presented with skin manifestations which proved to be histologically positive for Rosai–Dorfman disease. The patient clinically improved following the administration of intralesional steroids.

Hemosuccus pancreaticus (HP) is defined as bleeding from the ampulla of Vater through the pancreatic duct. It is a rare complication associated with acute or chronic pancreatitis. The source of bleeding can be from the pancreas itself or surrounding vessels, with the splenic artery most commonly involved. Diagnosing HP is challenging and computed tomography angiography remains the gold standard for diagnosis. We present the case of a 62-year-old male with recurrent pancreatitis complicated with HP. Imaging and endoscopy were consistent with bleeding from the section portion of the duodenum, which resolved without intervention.