The clinical and radiological features and prevalence of Neuro-Behçet's Disease: A retrospective cohort multicenter study in Saudi Arabia.

BACKGROUND: Neuro-Behçet's disease (NBD) is defined as primary neurological involvement in patients with systemic symptoms of BD. The variety of clinical presentations seen in NBD and the long list of similar conditions make diagnosis challenging. This retrospective study aimed to estimate the prevalence and describe neurological involvement in patients with Behçet's disease who presented to King Abdulaziz Medical Cities in Jeddah and Riyadh, Saudi Arabia. METHODS: This was a retrospective, cohort study which utilized a non-probability consecutive sampling technique to include all patients diagnosed with NBD patients. All patients with BD (215) were screened for neurological symptoms. Thirty-five patients were found to be diagnosed with NBD. Outcomes were estimated using the modified Rankin scale (mRS). RESULTS: In our cohort, one in six patients with BD was diagnosed with NBD. A total of 35 patients were diagnosed with NBD (mean age 27.56 ± 10.36 years; [2.88:1; Male: Female]). The main clinical features of NBD were headaches, weakness, unsteadiness, and dysarthria. The most commonly involved sites on imaging were the brainstem, diencephalon, cerebellum and basal ganglia. Oligocolonal bands were negative in all patients. Maintenance therapy most commonly included oral corticosteroids, azathioprine, and/or infliximab. Most patients received pulse corticosteroids alone when presenting with acute relapse. Half of our cohort was asymptomatic and three in four had favorable outcomes. CONCLUSION: NBD is common among patients with BD in our population with most patients having favorable outcomes. Patients might have a wide array of symptoms which might make the diagnosis challenging.

An Unusual Presentation of Large Glomangioma of the Hand.

Glomangiomas are rare and benign hamartomas that commonly occur in the upper extremities. It is not typical for benign glomangiomas to be larger than one centimeter in size, and they usually present as a faint, blue-red subungual papule associated with a triad of symptoms of paroxysmal pain, pain with cold exposure, and tenderness to touch. We herein report a case of a 72-year-old man with multiple comorbidities presented to our clinic as a case of right-hand middle finger swelling for the past five years. Initially, it was not painful. However, the pain became more noticeable when he lowers his hand, and it was relieved when he kept it elevated. There were no skin changes around it with minimal tenderness over the swelling. Hand MRI demonstrated a well-defined small lobulated nodule at the radial aspect of the middle finger, at the level of the middle phalanx with no invasion to an adjacent structure. Surgical excision was done and the patient was diagnosed by histopathology to have glomangioma. Glomangiomas, also known as glomus tumors, are rare and benign hamartomas that commonly occur in the upper extremities. The hand is the most common site for glomus tumors, particularly the subungual area, the lateral aspect of the digits, and the palms. Female patients are the most common to present with subungual glomangioma. Multiple papers reported different presentations, and due to the rarity of the conditions and overlapping in clinical and imaging characteristics with other conditions, it was challenging to diagnose. Such atypical cases must be approached with high clinical suspicion and proper imaging and investigations so as to not delay diagnosis and management.