Primary antiphospholipid syndrome manifesting as partial status epilepticus.

Primary antiphospholipid syndrome (APS) is an autoimmune disease defined by vascular thrombosis, pregnancy complications, and persistent antiphospholipid antibodies. Neurological manifestations include stroke, seizures, and chorea among others. Seizures are often precipitated by an acute ischemic event, but occasionally, structural abnormalities are absent. We present a 61-year-old man who developed partial seizures that progressed into partial status epilepticus. His seizures were intractable and required aggressive treatment with multiple anti-epileptic medications. He was diagnosed with primary APS and treated with anticoagulation. Head imaging did not reveal any acute ischemic events. This case demonstrates that primary APS may present as a refractory status epilepticus unrelated to acute cerebral ischemia.

Cervical artery dissection: risk factors, treatment, and outcome; a 5-year experience from a tertiary care center.

BACKGROUND: Cervical artery dissection (CAD) is a common cause of stroke in young individuals. Risk factors, treatment, and outcome of CAD are not well established. Our aim is to identify risk factors, outcome, and discuss treatment of CAD. METHODS: We reviewed medical records of patients seen at our institution between 2004 and 2009, using ICD-9 codes for CAD, for risk factors, treatment, type, and outcome. Poor outcome was defined as modified Rankin Scale (mRS) score within 6 months of >1 or recurrent stroke or transient ischemic attack (TIA). RESULTS: A total of 47 patients were identified. Twenty-four patients had spontaneous dissections (52%); 30 (63%) involved the carotid. As compared with spontaneous dissections, traumatic dissections occurred in younger patients (mean age 41.0 vs. 47.7 years, p = .064), were more likely to involve the vertebral artery (52% vs. 21%, p = .036, and caused less neurological deficits on presentation (48% vs. 75%, p = .075). Conversely, spontaneous dissections were more likely to occur in patients with hypertension (63% vs. 30%, p = .041) and hypercholesterolemia (46% vs. 9%, p = .008). Patients having no stroke had better outcomes (100% vs. 54%, p < .001). Anticoagulation was used in 17 patients (36%) and antiplatelets in 31 (64%). Outcome and adverse events were similar in the two groups. CONCLUSION: Hypertension and hypercholesterolemia were risk factors in spontaneous dissections, speaking for vascular wall abnormalities as potential contributors to pathophysiology of CAD. There was no evidence supporting one type of treatment over the other. A large ongoing prospective study should quell this controversy.

The value of sensory nerve conduction studies in the diagnosis of Guillain-Barré syndrome.

Electrophysiology plays a determinant role in Guillain-Barré syndrome (GBS) diagnosis, classification, and prognostication. However, traditional electrodiagnostic (EDX) criteria for GBS rely on motor nerve conduction studies (NCS) and are suboptimal early in the course of the disease or in the setting of GBS variants. Sensory nerve conduction studies, including the sural-sparing pattern and the sensory ratio are not yet included in EDX criteria despite their well-established role in GBS diagnosis. The aim of this review is to discuss the diagnostic value of sensory NCS in GBS, their role in establishing the diagnosis and predicting the outcome according to the various subtypes of the disease.

Autoimmune Brainstem Encephalitis: An Illustrative Case and a Review of the Literature.

Autoimmune brainstem encephalitis (BSE) is a rare neurological condition with a wide range of underlying etiologies. It can be subdivided into two broad groups: a primary inflammatory disease of the central nervous system (CNS) or a brainstem disorder secondary to systemic diseases where the CNS is only one of many affected organs. Symptoms range from mild to life-threatening manifestations. Most cases respond well to immunotherapy. Therefore, broad and in-depth knowledge of the various inflammatory disorders that target the brainstem is essential for guiding the diagnostic approach and assisting in early initiation of appropriate therapy. We herein report on a case of BSE and provide an overview of the various causes of autoimmune BSE with an emphasis on the clinical manifestations and diagnostic approach.