COVID-19-associated necrotizing encephalopathy presenting without active respiratory symptoms: a case report with histopathology.

Acute necrotizing encephalopathy (ANE) is a rare complication of coronavirus disease 2019 (COVID-19) secondary to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The condition is typically diagnosed based on characteristic neuroimaging findings in the context of active viral respiratory symptoms. We present a rare case of COVID-19-associated ANE presenting with expressive aphasia and encephalopathy in the absence of active respiratory symptoms. Initial evaluation revealed bilateral thalamic lesions and a mild neutrophilic-predominant pleocytosis on cerebrospinal fluid analysis, the latter of which has not been described in previously published cases. Presence of these atypical features prompted extensive diagnostic evaluation. Metagenomic next-generation sequencing on cerebrospinal fluid did not detect the presence of pathogenic nucleic acids. Thalamic biopsy revealed perivascular neutrophilic inflammation suggestive of small vessel vasculitis with surrounding hemorrhage and necrosis. Ultimately, the diagnosis was made following detection of SARS-CoV-2 serologies and after exclusion of alternative etiologies. The patient was successfully treated with a short course of high-dose methylprednisolone with favorable outcome.

Management of Ischemic Stroke Following Left Ventricular Assist Device.

BACKGROUND: Acute ischemic stroke is a common complication and an important source of morbidity and mortality in patients with left ventricular assist devices. There are no standardized protocols to guide management of ischemic stroke among patients with left ventricular assist device. We evaluated our experience treating patients who had an acute ischemic stroke following left ventricular assist device placement. METHODS: We retrospectively reviewed all patients who underwent left ventricular assist device placement from 2010-2019 and identified patients who had acute ischemic stroke following left ventricular assist device placement. RESULTS: Of 216 patients having left ventricular assist device placement (mean±SD age 52.9±16.2 years, women 26.9%), 19 (8.8%) had acute ischemic stroke (mean±SD age 55.8±12.0 years, women 36.8%). Median (interquartile range) time to ischemic stroke following left ventricular assist device placement was 96 (29-461) days. At the time of the ischemic stroke, 16/19 (84.2%) patients were taking both antiplatelet and anticoagulation therapy, 1/19 (5.3%) patient was receiving only anticoagulants, 1/19 (5.3%) patient was taking aspirin and dipyridamole, and 1/19 (5.3%) patient was not taking antithrombic agents. INR was subtherapeutic (INR<2.0) in 7/17 (41.2%) patients. No patient was eligible to receive thrombolytic therapy, while 5/19 (26.3%) underwent mechanical thrombectomy. Anticoagulation was continued in the acute stroke phase in 11/19 (57.9%) patients and temporarily held in 8/19 (42.1%) patients. Hemorrhagic transformation of the ischemic stroke occurred in 6/19 (31.6%) patients. Anticoagulation therapy was continued following ischemic stroke in 4/6 (66.7%) patients with hemorrhagic transformation. CONCLUSIONS: While thrombolytic therapy is frequently contraindicated in the management of acute ischemic stroke following left ventricular assist device, mechanical thrombectomy remains a valid option in eligible patients. Anticoagulation is often continued through the acute phase of ischemic stroke secondary to concerns for LVAD thrombosis. The risks and benefits of continuing anticoagulation must be weighed carefully, especially in patients with large infarct volume, as hemorrhagic transformation remains a common complication.

Novel technique for trialing peripheral nerve stimulation: ultrasonography-guided StimuCath trial.

OBJECTIVE Peripheral nerve stimulation (PNS) has been used for the treatment of neuropathic pain for many decades. Despite the specific indications for PNS, clinicians often have difficulty screening for candidates likely to have a good or fair outcome. Given the expense of a permanent implant, most insurance companies will not pay for the implant without a successful PNS trial. And since PNS has only recently been approved by the US Food and Drug Administration, many insurance companies will not pay for a conventional trial of PNS. The objective of this study is to describe a short low-cost method for trialing and screening patients for peripheral nerve stimulator implantation. Additionally, this study demonstrates the long-term efficacy of PNS in the treatment of chronic neuropathic pain and the relative effectiveness of this novel screening method. METHODS The records of all patients who had undergone trialing and implantation of a PNS system for chronic refractory pain at the authors' institution over a 1-year period (August 1, 2012-July 31, 2013) were examined in this retrospective case series. The search revealed 17 patients, 13 who had undergone a novel in-office ultrasonography-guided StimuCath screening technique and 4 who had undergone a traditional week-long screening procedure. All 17 patients experienced a successful PNS trial and proceeded to permanent PNS system implantation. Patients were followed up for a mean duration of 3.0 years. Visual analog scale (VAS) pain scores were used to assess pain relief in the short-term (< 6 weeks), at 1 year, and at the last follow-up. Final outcome was also characterized as good, fair, poor, or bad. RESULTS Of these 17 patients, 10 were still using their stimulator at the last follow-up, with 8 of them obtaining good relief (classified as ≥ 50% pain relief, with an average 81% reduction in the VAS score) and 2 patients attaining fair relief (< 50% relief but still using stimulation therapy). Among the remaining 7 patients, the stimulator had been explanted in 4 and there had been no relief in 3. Excluding explanted cases, follow-up ranged from 14 to 46 months, with an average of 36 months. Patients with good or fair relief had experienced pain prior to implantation for an average of 5.1 years (range 1.8-15.2 years). A longer duration of pain trended toward a poorer outcome (bad outcome 7.6 years vs good outcome 4.1 years, p = 0.03). Seven (54%) of the 13 patients with the shorter trial experienced a good or fair outcome with an average 79% reduction in the VAS score; however, all 4 of the bad outcome cases came from this group. Three (75%) of the 4 patients with the longer trial experienced a good or fair outcome at the last follow-up, with an average 54% reduction in the VAS score. There was no difference between the trialing methods and the proportion of favorable (good or fair) outcomes (p = 0.71). CONCLUSIONS Short, ultrasonography-guided StimuCath trials were feasible in screening patients for permanent implantation of PNS, with efficacy similar to the traditional week-long screening noted at the 3-year follow-up.

Neuropathology of Congenital Heart Disease in an Inpatient Autopsy Cohort 2000-2017.

Background As a result of medical and surgical advancements in the management of congenital heart disease (CHD), survival rates have improved substantially, which has allowed the focus of CHD management to shift toward neurodevelopmental outcomes. Previous studies of the neuropathology occurring in CHD focused on cases preceding 1995 and reported high rates of white matter injury and intracranial hemorrhage, but do not reflect improvements in management of CHD in the past 2 decades. The purpose of this study is therefore to characterize the neuropathological lesions identified in subjects dying from CHD in a more-recent cohort from 2 institutions. Methods and Results We searched the autopsy archives at 2 major children's hospitals for patients with cyanotic congenital cardiac malformations who underwent autopsy. We identified 50 cases ranging in age from 20 gestational weeks to 46 years. Acquired neuropathological lesions were identified in 60% (30 of 50) of subjects upon postmortem examination. The most common lesions were intracranial hemorrhage, most commonly subarachnoid (12 of 50; 24%) or germinal matrix (10 of 50; 20%), hippocampal injuries (10 of 50; 20%), and diffuse white matter gliosis (8 of 50; 16%). Periventricular leukomalacia was rare (3 of 50). Twenty-six subjects underwent repair or palliation of their lesions. Of the 50 subjects, 60% (30 of 50) had isolated CHD, whereas 24% (12 of 50) were diagnosed with chromosomal abnormalities (trisomy 13, 18, chromosomal deletions, and duplications) and 16% (8/50) had multiple congenital anomalies. Conclusions In the modern era of pediatric cardiology and cardiac surgery, intracranial hemorrhage and microscopic gray matter hypoxic-ischemic lesions are the dominant neuropathological lesions identified in patients coming to autopsy. Rates of more severe focal lesions, particularly periventricular leukomalacia, have decreased compared with historical controls.

Acute, Nontraumatic Spontaneous Spinal Subdural Hematoma: A Case Report and Systematic Review of the Literature.

Spontaneous spinal subdural hematoma (sSDH) is a rare condition outright. Moreover, cases that occur spontaneously in the absence of an identifiable etiology are considerably less common and remain poorly understood. Here, we present the case of a 43-year-old man with spontaneous sSDH presenting with acute onset low back pain and paraplegia. Urgent magnetic resonance imaging identified a dorsal SDH from T8 to T11 with compression of the spinal cord. Emergent T8-T10 laminectomies with intradural exploration and hematoma evacuation were performed. However, despite prompt identification and appropriate action, the patient's recovery was modest and significant disability remained at discharge. This unique and unusual case demonstrates that spontaneous sSDH requires prompt surgical treatment to minimize associated morbidity and supports the association between the presence of severe neurological deficits upon initial presentation with less favorable outcomes. We performed a comprehensive systematic review of spontaneous sSDH of unknown etiology, which demonstrates that emergent surgical intervention is indicated for patients presenting with severe neurological deficits and the presence of these deficits is predictive of poor neurological outcome. Furthermore, conservative management should be considered in patients presenting with mild neurological deficits as spontaneous resolution followed by favorable neurological outcomes is often observed in these patients.

Value of Targeted Epidural Blood Patch and Management of Subdural Hematoma in Spontaneous Intracranial Hypotension: Case Report and Review of the Literature.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a more common than previously noted condition (1-2.5 per 50,000 persons) typically caused by cerebrospinal fluid (CSF) leakage. Initial treatment involves conservative therapies, but the mainstay of treatment for patients who fail conservative management is the epidural blood patch (EBP). Subdural hematoma (SDH) is a common complication occurring with SIH, but its management remains controversial. METHODS: In this report, we discuss a 62-year-old woman who presented with a 5-week history of orthostatic headaches associated with nausea, emesis, and neck pain. Despite initial imaging being negative, the patient later developed classic imaging evidence characteristic of SIH. Magnetic resonance imaging was unrevealing for the source of the CSF leak. Radionuclide cisternography showed possible CSF leak at the right-sided C7-T1 nerve root exit site. After failing a blind lumbar EBP, subsequent targeted EBP at C7-T1 improved the patient's symptoms. Two days later she developed a new headache with imaging evidence of worsening SDH with midline shift requiring burr hole drainage. This yielded sustained symptomatic relief and resolution of previously abnormal imaging findings at 2-month follow-up. RESULTS: A literature review revealed 174 cases of SIH complicated by SDH. This revealed conflicting opinions concerning the management of this condition. CONCLUSIONS: Although blind lumbar EBP is often successful, targeted EBP has a lower rate of patients requiring a second EBP or other further treatment. On the other hand, targeted EBP has a larger risk profile. Depending on the clinic situation, treatment of the SDH via surgical evacuation may be necessary.

Epidural Spinal Cord Stimulation: A Novel Therapy in the Treatment of Restless Legs Syndrome.

BACKGROUND: We report a unique finding of a patient whose restless legs syndrome (RLS) symptoms abated after the placement of a spinal cord stimulator for chronic neuropathic pain. RLS is a common disorder, with many patients unable to find sufficient relief from their symptoms. CASE DESCRIPTION: A patient diagnosed with neuropathic pain who also suffered from RLS symptoms despite medication therapy underwent implantation of a spinal cord stimulator after a successful trial. This patient was interviewed formally about his RLS symptoms immediately before his procedure and at 6 weeks, 6 months, and 2.5 years after the procedure. The patient also completed the International Restless Legs Syndrome Scale questionnaire to objectively quantify the severity of his symptoms. Finally, the patient kept a 5-day journal detailing when the stimulator was in use. The patient reported subjective symptomatic improvement in his RLS symptoms with improved sleep quality and quantity, in addition to improvement in his back pain. The patient's score on the International Restless Legs Syndrome Scale improved after implantation from 33 to 0 on a 40-point scale. Moreover, when asked to keep a journal record of his stimulator use, the patient noted that he only used the stimulator before going to bed to help his RLS symptoms and no longer required any medication for his previous RLS symptoms. CONCLUSIONS: Epidural stimulation may be an additional, alternative, or novel therapy in the treatment of RLS.