The artery of Percheron (AOP) is a variant of the paramedian thalamic vasculature that supplies blood to the medial aspect of the thalamus and the rostral midbrain. The presentation of an infarct in this territory varies widely and is often characterized by nonspecific neurological deficits, with altered mental status, decreased level of consciousness, and memory impairment being among the most common. AOP infarcts are often missed on initial computed tomography (CT) scan, and additional imaging is usually not done due to low suspicion for stroke in most cases. There have been an increasing number of reports of AOP infarction, illustrating the diversity of clinical presentations and the challenge this presents to clinicians in the acute setting. Lacking the classic signs of stroke, many of these patients experience a delay in recognition and treatment, with the majority of diagnoses occurring outside the tissue plasminogen activator (tPA) window. This case highlights the unusual presentation and diagnostic difficulty of a patient with an AOP infarct, and serves as a reminder to include thalamic pathology in patients presenting with vague neurological symptoms and no obvious signs of stroke.

Anti-glutamic acid decarboxylase (GAD) antibodies have been discovered in a variety of neurological syndromes with unique presentations. These syndromes include limbic encephalitis (LE), stiff person syndrome (SPS), opsoclonus-myoclonus-ataxia syndrome, cerebellar ataxia, status epilepticus, and palatal myoclonus among others. We present two patients who presented with Guillain-Barré (GBS) and myasthenia gravis (MG) like syndromes, who were found to have anti-GAD antibodies. These case reports highlight the complex presentation of patients with neurological disorders associated with anti-GAD antibodies. The proper identification of anti-GAD antibody’s presence has proven to be beneficial in treatment and provide enhanced quality of life.

Leber hereditary optic neuropathy (LHON) is a genetic condition that typically presents with unilateral, painless, sub-acute central vision loss followed by contralateral vision loss after a few weeks to months. It is a rare disease that typically affects young adults-men more than women-and is a relatively common cause of blindness. It is due to a mutation in mitochondrial DNA (mtDNA). The majority (more than 95%) of patients have one of three mtDNA point mutations: m.14484T→C, m.3460G→A, or m.11778G→A. These mutations lead to disruption of the mitochondrial respiratory chain activating pro-apoptotic pathways. For reasons unknown, this insult tends to affect the retinal ganglion cells more than any other cell in the body, leading to the disease state. Due to its low prevalence in the United States (1:50,000), this diagnosis is often overlooked, misdiagnosed, and mismanaged, which may exacerbate symptoms. It is essential then for physicians to recognize the presentation of and understand the diagnostic work-up for LHON. In this case report, we present the diagnostic challenges of a patient who presented with progressive vision loss, discuss the various differential diagnoses, review the literature on LHON, and propose an explanatory model for vision loss in patients with LHON.

Moyamoya disease, well described in literature, is a chronic cerebrovascular occlusive disorder. It is characterized by progressive stenosis/occlusion of the terminal portions of the internal carotid arteries (ICA) and the proximal portions of the middle cerebral arteries (MCA). Less frequently described is Moyamoya syndrome, the name given to radiographic findings consistent with Moyamoya disease, but with an identifiable cause. The diseases associated with Moyamoya Syndrome include Sickle Cell Disease (SCD), Thalassemias, and Down's Syndrome to name a few. Common complications of Moyamoya include both ischemic and hemorrhagic strokes. Upon literature review, Moyamoya syndrome caused by SCD is not well described. When it is, the discussion is centered around the pediatric patient population and surgical management. Our case report describes a 22-year-old African American female with SCD who initially presented with Acute Chest Syndrome. Her hospital course was complicated by development of overt debilitating neurologic deficits. Subsequently, she was found to have Moyamoya Syndrome on neuroimaging. She was successfully treated with medical management without any surgical intervention. This case highlights the necessity of thorough examination, differential diagnosis, imaging findings, and consideration of predisposing syndromes in the work-up for Moyamoya syndrome; especially individuals with Sickle Cell Disease. Disclosures No relevant conflicts of interest to declare.

Idiopathic basal ganglia calcification (IBGC), commonly referred to as Fahr’s disease, is a rare neurological disorder characterized by the abnormal, symmetrical, and bilateral calcification of the basal ganglia and other brain regions. Patients typically present in their forties and fifties with various neurologic and/or psychiatric symptoms, including movement disorders, Parkinsonism, psychosis, and depression.The pathophysiology of this disease is not completely understood; however, several gene mutations have been identified in the pathogenesis of Fahr’s disease. These mutations display an autosomal dominant inheritance pattern. Furthermore, the regional phenotypic expression of calcifications differs greatly from patient to patient, as do their clinical presentations. Here, we describe a patient who presented with psychiatric manifestations and imaging consistent with Fahr’s disease.