Anomia in a Lung Transplant Recipient

Progressive multifocal leukoencephalopathy (PML) is a rare, fatal sub-acute demyelinating disease of subcortical white matter most commonly caused by reactivation of the ubiquitous Jon Cunningham virus (JCV). PML has been documented in patients with immune-suppressed conditions, including but not limited to organ transplantation, HIV/AIDS, malignancy, and auto-immune diseases. We describe the case of a 65-year old male who presented with anomia, confusion, left upper extremity tremor, and generalized weakness 3 years after undergoing bilateral lung transplantation for idiopathic pulmonary fibrosis. Cerebrospinal fluid (CSF) and MRI findings confirmed the diagnosis of PML. We also review other reported cases as well as available treatment modalities for this rare condition. Lung transplantation has become the standard of care in patients with end-stage chronic respiratory failure. As the prevalence of lung transplantation increases worldwide, more data is becoming available regarding long-term outcomes. Our findings suggest that neurological symptoms and white matter changes on imaging should prompt high clinical suspicion for PML in this patient population.https://scholarlycommons.henryford.com/merf2020caserpt/1121/thumbnail.jp

Eagle Syndrome Presenting in a Patient as Dysphagia and Failed Intubations

Eagle syndrome is a rare elongation of the styloid process or calcification of the stylohyoid ligament. Most cases are asymptomatic but compression on head and neck structures can result in dysphagia and neck pain. Etiology may be related to surgical scar tissue but is unclear due to limited case presentation. A 52 year-old male with surgical history of tonsillectomy presented to the hospital with shortness of breath. He developed sudden onset dyspnea and pink frothy sputum. He desaturated and was put on BiPaP in the ED. Chest X-ray revealed flash pulmonary edema and he was admitted for hypoxic respiratory failure. Within three days his status was complicated by bacteremia and atrial fibrillation and cardiology recommended a trans-esophageal echo before discharge. He was unable to tolerate TEE at the bedside and anesthesia with intubation was planned instead. Three intubation attempts failed despite fiberoptic and glidescope assistance and limited neck and laryngeal tissue mobility were noted. Follow-up imaging of CT neck/chest without contrast showed complete calcified left stylohyoid ligament extending from the styloid process to the hyoid bone. Post-procedure he endorsed dysphagia with both solids and liquids and voice change. Swallow studies and flexible laryngoscopy showed oropharyngeal dysphagia and silent aspiration with nectar thick liquids. He has not been able to tolerate NGT and has been placed on TPN pending gastrostomy tube. This case shows a rare symptomatic presentation of Eagle Syndrome with CT visualization and contributes to gathering more information as to the etiology and clinical presentation of the condition.https://scholarlycommons.henryford.com/merf2020caserpt/1026/thumbnail.jp

Palliative care in brain tumors

The diagnosis of a brain tumor is a life-changing event for patients and families. High-grade gliomas (especially glioblastomas) are incurable, and long-term survival is limited. Metastatic brain lesions comprise the majority of intracranial neoplasms and are a significant source of morbidity and mortality in patients with systemic cancer. Response to treatment, prognosis, and survival depends not only on the underlying pathology, but more importantly on recently defined molecular features. Other crucial predictors of survival include age and performance status. Among patients with primary brain tumors, neurologic decline and psychological distress contribute to a high symptom burden and impaired quality of life (QoL) throughout the disease trajectory. While many symptoms in central nervous system (CNS) and non-CNS cancers overlap, others predominate in the brain tumor population, including seizures, headaches, depression, fatigue, and treatment-induced toxicity, all of which can be addressed with palliative interventions. Patients, families, and caregivers also report disproportionately high supportive care needs, which frequently differ from those of other systemic cancers. In addition, progressive neurologic decline often results in impaired communication and decision-making capacity at the end of life. Early palliative care (PC) integration has become more common in systemic cancers, but remains limited in neuro-oncology. These factors combined contribute to a uniquely challenging disease course that may benefit from a multidisciplinary approach with early involvement of specialized (PC) to address tumor-related symptoms and improve QoL. We review how to approach patients with brain tumors and address prognosis, symptom management, and advance care planning with the goal of improving QoL for patients, families, and caregivers

Neuro-oncology and supportive care: the role of the neurologist

The diagnosis of a brain tumor is a life-changing event for patients and their families. Despite numerous treatment advances, malignant brain tumors are universally incurable and long-term survival is limited. Treatment response, prognosis, and survival depend on underlying histopathology and recently defined molecular features. Patients suffer from a disproportionately high symptom burden throughout the disease trajectory and at the end of life. Pronounced neurologic decline and psychological distress significantly impair quality of life (QoL) and impose high supportive care needs relative to other systemic cancers. Palliative interventions addressing brain tumor-specific symptoms, such as seizures, cognitive dysfunction, and headaches, are paramount to maintaining QoL. In the terminal phase of illness, most brain tumor patients lose the ability to communicate and participate in end-of-life decision-making. The benefits of advance care planning and early integration of specialized palliative care are well-established in other systemic cancers and have received wider recognition in neuro-oncology. We review how to approach neurological symptoms in brain tumor patients, as well as address prognosis and advance care planning with the goal of improving QoL for patients and caregivers

Cytochrome P450 metabolites of arachidonic acid are elevated in stroke patients compared with healthy controls

CYP450AAM [arachidonic acid metabolites of the CYP450 (cytochrome P450) enzyme system] have a range of biological functions. CYP450AAM are involved in the pathogenesis of hypertension, renal function and vascular function, yet their role in stroke has not been clarified. We aimed at determining the levels of circulating CYP450 metabolites in patients with acute ischaemic stroke (<96 h) compared with healthy age- and gender-matched controls. This was a retrospective case-controlled study of 44 acute ischaemic stroke patients and 44 matched controls. A subset of acute ischaemic stroke patients was available for follow-up. Acute ischaemic stroke patients had elevated plasma CYP450AAM, including 20-HETE (20-hydroxyeicosatetraenoic acid) (1921±170 compared with 1108±170 pmol/l, P<0.001), EETs (epoxyeicosatrienoic acids) (77.88±3.34 compared with 35.35±3.34 nmol/l, P<0.0001) and DiHETEs (dihydroxyeicosatetraenoic acids) (92.87±4.61 compared with 68.17±4.61 nmol/l, P<0.0001), as well as increased plasma F2-isoprostane levels (3754±538 compared with 1947±538 pmol/l, P<0.02), the latter a marker of oxidative stress, compared with controls. In a subset analysis of the stroke patients, plasma 20-HETE, EETs and F2-isoprostanes were attenuated 30 days after the stroke. Baseline 20-HETE levels were also associated with lesion size and functional indices within the stroke patients. The present study highlights the elevation in CYP450AAM and oxidative stress in acute ischaemic stroke patients. Further investigation of the effect this has on long-term clinical outcome or whether this can be modified by treatment is warranted