A Rare Presentation of a Complex Mixed Autoimmune Encephalitis Diagnosis: A Case Report and Literature Review.

This case report presents a unique case of a difficult differential diagnosis of autoimmune encephalitis (AE) in the setting of Mycoplasma pneumoniae. A 40-year-old female with a history of Hashimoto thyroiditis, polycystic ovarian syndrome, and a lower respiratory infection presented to the emergency department with new-onset progressive neurological symptoms. These included generalized tonic-clonic seizure and worsening respiratory status that required intubation and tracheostomy. Blood cultures returned positive for M. pneumoniae. We concluded this to be a mixed diagnosis case of anti-glutamic acid decarboxylase 65 (anti-GAD65), Bickerstaff's brainstem encephalitis (BBE), Hashimoto's encephalopathy (HE), and Miller Fisher Syndrome (MFS) concurrently in the setting of M. pneumoniae. Initial treatment with intravenous immunoglobulin showed minimal improvement; however, subsequent treatment with plasmapheresis proved to be beneficial for the patient. Over the course of the plasma exchange therapy (PLEX), the patient slowly became more alert, attentive, and verbal. She was able to answer simple questions and follow commands. Common trends of age, gender, presenting symptoms, associated antibodies, and sessions of PLEX in different AE diseases were identified through a literature review. Only 69.7% of the cases implemented PLEX or plasmapheresis. Currently, there is no standard protocol for the treatment of AE. Our case report aims to present a clinically complicated example of AE and to provide further evidence to support PLEX as an important therapeutic option.

Historical and projected public spending on drugs for rare diseases in Canada between 2010 and 2025.

OBJECTIVE: Rare diseases are life-threatening, debilitating, or serious chronic conditions that affect < 50/100,000 people. Canadians can only access approximately 60% of drugs for rare diseases (DRDs), which is partially related to high per-patient costs and payers' affordability concerns. However, limiting access to DRDs can reduce survival and quality of life among patients and caregivers. Therefore, we projected Canadian non-oncology DRD spending relative to total public drug spending to provide perspective for decision makers. METHODS: Candidate historical (2010-2020) and pipeline (2021-2025) Canadian-marketed non-oncology DRDs were identified using definitions from the European Medicines Agency and the US Food and Drug Administration databases. Inclusion and exclusion criteria were applied to identify eligible DRDs. Public payer claims data, prevalence rates, regulatory, and health technology assessment factors were used to project DRD spending in relation to total Canadian public drug spending. RESULTS: We included 42 historical DRDs and 122 pipeline DRDs. Public spending on DRDs grew from $14.8 million in 2010 (11 DRDs) to $380.9 million in 2020, then a projected $527.6 million in 2021 (59 potential DRDs) and $1.6 billion in 2025 (164 potential DRDs). Projected DRD spending increased from 3.2% of $16.5 billion public drug spending in 2021 to 8.3% of $19.4 billion in 2025. These projections do not include confidential manufacturer discounts, health outcome-related offsets, or additional safety-related costs. CONCLUSIONS: Projected DRD spending shows robust growth but remains a fraction of total public drug spending. Limiting DRD access because of this growth is not aligned with Canadian patient or societal values. Given the renewed interest in a Canadian DRD framework, our results may help guide discussions that aim to balance control of public drug spending with the well-being of patients with rare diseases.

Corkscrew Angiopathy of the Basilar Artery: A Case Report.

A vascular malformation is an abnormal development of blood vessels that can be found in arteries, veins, or the lymphatic system. While pathology such as aneurysms, arteriovenous malformations (AVMs), dissections, and dolichoectasia have been documented frequently and linked to an increased incidence of stroke, we present a rare finding of a 'corkscrew' appearance of the basilar artery causing recurrent posterior circulation symptoms. Imaging during 3 separate hospitalizations showed recurrent acute strokes within the basilar artery vascular territory. A conventional cerebral angiogram revealed a corkscrew appearance of the basilar artery with no dilatation, outpouching, or aneurysm.

Minimal evidence that untreated psychosis damages brain structures: a systematic review.

INTRODUCTION: A longer duration of untreated psychosis (DUP) is associated with poor outcomes in first-episode psychosis (FEP); however, it is unclear whether this is due to the effects of psychosis on brain structure. We systematically reviewed the literature on the association between the length of untreated psychosis and brain structure in first-episode psychosis. METHODS: We searched three electronic databases and conducted forward and backward citation searching to identify relevant papers. Studies were included if they: (1) included patients with a psychotic disorder who were treatment naïve or minimally treated; and (2) had correlated measures of DUP or duration of untreated illness (DUI) with structural measures. RESULTS: We identified 48 studies that met the inclusion criteria. Forty-three examined the correlation between DUP and brain structure, and 19 examined the correlation between DUI and brain structure. There was evidence of significant associations in brain regions considered important in psychosis; however, the proportion of significant associations was low and the findings were inconsistent across studies. The majority of included studies were not primarily designed to examine whether DUP/DUI is correlated with brain structure, and there were methodological limitations in many studies that prevent drawing a strong conclusion. CONCLUSION: To date, there is minimal evidence of an association between untreated psychosis and brain structure in FEP. Although the body of literature is substantial, there are few hypothesis-driven studies with a primary objective to answer this question. Future studies should be specifically designed to examine whether untreated psychosis has a deleterious effect on brain structure.