Improving Outcome in Severe Myasthenia Gravis and Guillain-Barré Syndrome.

When progressive and severe, myasthenia gravis and Guillain-Barré syndrome may have the potential for fatal and unfavorable clinical outcomes. Regardless of important differences in their clinical course, the development of weakness of oropharyngeal muscles and respiratory failure with requirement of mechanical ventilation is the main driver of poor prognosis in both conditions. The need for prolonged mechanical ventilation is particularly relevant because it immobilizes the patient and care becomes extraordinarily complex due to daily risks of systemic complications. Additionally, patients with myasthenia gravis often require long-term immunosuppressive treatments with associated toxicity and infectious risks. Unlike myasthenia gravis, the recovery period is prolonged in Guillain-Barré syndrome, but often favorable, even in the more severely affected patients. Outcome, for a large part, is determined by expert neurocritical care.

Locked in from fulminant GBS after lumbar spine surgery.

Guillain-Barré syndrome (GBS) describes a neurological syndrome characterised by acute, areflexic paralysis, often preceded by an immune stimulating event such as infection or surgery. Spinal surgery as an inciting event is very uncommon with few reported cases. When paraparesis develops in close proximity of surgery, surgical complications should be considered, but if an asymptomatic clinical interval precedes a progressive ascending weakness this association weakens and may support an immunological mechanism. GBS after lumbar surgery is wholly unexpected and thus there are significant challenges in recognising and making the diagnosis. We report a case of fulminant GBS that progressed to loss of all motor function following elective lumbar spine surgery.

Variable Significance of Brain MRI Findings in Infective Endocarditis and Its Effect on Surgical Decisions.

OBJECTIVE: To determine how brain magnetic resonance imaging (MRI) findings impact clinical outcomes in patients with infective endocarditis (IE) and to propose a management algorithm for patients with neurologic symptoms who are candidates for valve surgery (VS). PATIENTS AND METHODS: Data from our center were retrospectively reviewed for patients hospitalized with IE between January 1, 2007, and December 31, 2014. Outcomes were postoperative intracerebral hemorrhage (ICH), 6-month mortality, and functional outcome at last follow-up as described by the modified Rankin Scale (mRS) score. Good outcome was defined as an mRS score of 2 or less. RESULTS: A total of 361 patients with IE were identified, including 127 patients (35%) who had MRI. One hundred twenty-six of 361 patients (35%) had neurologic symptoms, which prompted MRI in 79 of 127 patients (62%); 74 of 79 (94%) had acute or subacute MRI abnormalities. One patient with subarachnoid and multifocal ICH on MRI developed postoperative ICH. Patients with VS despite MRI abnormalities had lower 6-month mortality (odds ratio [OR], 0.17; 95% CI, 0.06-0.48; P<.001) and better functional outcome (OR, 4.43; 95% CI, 1.51-13.00; P=.005). Irrespective of VS, lobar or posterior fossa ICH on MRI was associated with 6-month mortality (OR, 3.58; 95% CI, 1.22-10.50; P=.02) and territorial ischemic stroke was inversely associated with good mRS (OR, 0.29; 95% CI, 0.13-0.66; P=.002). In neurologically asymptomatic patients who had VS, MRI findings did not impact 6-month mortality or functional outcomes. CONCLUSION: Magnetic resonance imaging detects a large number of abnormalities in patients with IE. Preoperative lobar hematoma and large territorial stroke determine outcome irrespective of VS. When indicated, VS increases the odds of a good outcome despite MRI abnormalities.

Stomaching Acute Brain Injury.

Gastrointestinal complications, including hemorrhage, can occur with intracranial lesions and after craniotomy. As early as the 19th century, surgeons were aware that brain tumors could cause gastric ulcers and acute perforations. Investigators used animal experiments both to reproduce these clinical observations and to seek ways to block the effects. Gastrointestinal lesions were seen as a result of the stress of acute brain injury or as a direct consequence of brain surgery. The thinking at the time was markedly influenced by the presumed stress and psychic factors proposed by Cannon. This historical vignette summarizes the major experimental works linking the brain with the stomach.

Characteristics of Spontaneous Spinal Cord Infarction and Proposed Diagnostic Criteria.

Importance: Spinal cord infarction (SCI) is often disabling, and the diagnosis can be challenging without an inciting event (eg, aortic surgery). Patients with a spontaneous SCI are often misdiagnosed as having transverse myelitis. Diagnostic criteria for SCI are lacking, hindering clinical care and research. Objective: To describe the characteristics of spontaneous SCI and propose diagnostic criteria. Design, Setting, and Participants: An institution-based search tool was used to identify patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1997 to December 2017 with a spontaneous SCI. Patients provided written consent to use their records for research. Participants were 18 years and older with a diagnosis of spontaneous SCI (n = 133), and controls were selected from a database of alternative myelopathy etiologies for validation of the proposed diagnostic criteria (n = 280). Main Outcomes and Measures: A descriptive analysis of SCI was performed and used to propose diagnostic criteria, and the criteria were validated. Results: Of 133 included patients with a spontaneous SCI, the median (interquartile range) age at presentation was 60 (52-69) years, and 101 (76%) had vascular risk factors. Rapid onset of severe deficits reaching nadir within 12 hours was typical (102 [77%]); some had a stuttering decline (31 [23%]). Sensory loss occurred in 126 patients (95%), selectively affecting pain/temperature in 49 (39%). Initial magnetic resonance imaging (MRI) spine results were normal in 30 patients (24%). Characteristic MRI T2-hyperintense patterns included owl eyes (82 [65%]) and pencil-like hyperintensity (50 [40%]); gadolinium enhancement (37 of 96 [39%]) was often linear and located in the anterior gray matter. Confirmatory MRI findings included diffusion-weighted imaging/apparent diffusion coefficient restriction (19 of 29 [67%]), adjacent dissection/occlusion (16 of 82 [20%]), and vertebral body infarction (11 [9%]). Cerebrospinal fluid showed mild inflammation in 7 of 89 patients (8%). Diagnostic criteria was proposed for definite, probable, and possible SCI of periprocedural and spontaneous onset. In the validation cohort (n = 280), 9 patients (3%) met criteria for possible SCI, and none met criteria for probable SCI. Conclusions and Relevance: This large series of spontaneous SCIs provides clinical, laboratory, and MRI clues to SCI diagnosis. The diagnostic criteria proposed here will aid clinicians in making the correct diagnosis and ideally improve future care for patients with SCI. The validation of these criteria supports their utility in the evaluation of acute myelopathy.

Clinical factors associated with Guillain-Barré syndrome following surgery.

BACKGROUND: We sought to identify clinical associations and potential triggers of Guillain-Barré syndrome (GBS) within 6 weeks of surgery. METHODS: We retrospectively reviewed consecutive patients diagnosed with GBS within 6 weeks of a surgery between January 1995 and June 2014 at Mayo Clinic. Postsurgical GBS was defined as symptom onset within 6 weeks of surgery. Patients with postsurgical GBS were compared with patients who did not have a surgery prior to GBS onset to determine differences between groups. RESULTS: A total of 208 patients with GBS, median age 55 years (interquartile range [IQR] 41-68), were included. Nineteen patients (9.1%) developed postsurgical GBS. Median duration from the surgery to onset of first GBS symptom was 15 days (IQR 9-37). The main types of surgeries preceding GBS were gastrointestinal, orthopedic, and cardiac. General anesthesia was used in 18 (95%) and conscious sedation in 1 (5%) patient. Among the 19 patients with postsurgical GBS, 11 (57.9%) had a known diagnosis of malignancy. Autoimmune conditions were present in 5 (26.3%) patients. Postoperative infection was found in 4 (21%) patients. On univariate analysis, the factors that showed an association with postsurgical GBS were age (p = 0.02), malignancy (p ≤ 0.0004), active malignancy (p = 0.03), preexisting autoimmune disorder (p = 0.02), and infection (p = 0.0001). On multivariate analysis, only active malignancy (0.03) remained associated. CONCLUSIONS: Surgery antedated GBS in 9.1% of patients. Postsurgical GBS was more common in patients with an active malignancy. A prospective study is needed to determine whether active malignancy represents an independent risk factor for the development of postsurgical GBS.

Spinal cord infarction: Clinical and imaging insights from the periprocedural setting.

OBJECTIVE: Describe the range of procedures associated with spinal cord infarction (SCI) as a complication of a medical/surgical procedure and define clinical and imaging characteristics that could be applied to help diagnose spontaneous SCI, where the diagnosis is often less secure. METHODS: We used an institution-based search tool to identify patients evaluated at Mayo Clinic, Rochester, MN from 1997 to 2016 with a periprocedural SCI. We performed a descriptive analysis of clinical features, MRI and other laboratory findings, and outcome. RESULTS: Seventy-five patients were identified with SCI related to an invasive or non-invasive surgery including: aortic aneurysm repair (49%); other aortic surgery (15%); and a variety of other procedures (e.g., cardiac surgery, spinal decompression, epidural injection, angiography, nerve block, embolization, other vascular surgery, thoracic surgery) (36%). Deficits were severe (66% para/quadriplegia) and maximal at first post-procedural evaluation in 61 patients (81%). Impaired dorsal column function was common on initial examination. Imaging features included classic findings of owl eyes or anterior pencil sign on MRI (70%), but several other T2-hyperintensity patterns were also seen. Gadolinium enhancement of the SCI and/or cauda equina was also common when assessed. Six patients (10%) had an initial normal MRI despite a severe deficit. CONCLUSIONS: Procedures associated with SCI are many, and this complication does not exclusively occur following aortic surgery. The clinical and radiologic findings that we describe with periprocedural SCI may be used in future studies to help distinguish spontaneous SCI from alternate causes of acute myelopathy.

On Aneurysmal Rupture and Rerupture.

A warning leak is a curious phenomenon attributed to cerebral aneurysms. Once the leak occurs, it has been postulated it could lead to a more catastrophic rebleeding. The designation "warning leak" trickled into neurosurgery vocabulary as early as the 1950s. The phenomenon has been poorly understood and characterized, but its presence spurs emergency physicians and neurointensivists to take action to secure the aneurysm. Rapid treatment of a recently discovered aneurysm is now commonplace, but it has not always been so. Antifibrinolytic agents spawned particular interest in the late 1970s, when many neurosurgeons postponed surgery after a recent hemorrhage. This historical vignette reviews the early views on aneurysmal rupture, rerupture, and the role of fibrinolysis.

Propofol Frenzy: Clinical Spectrum in 3 Patients.

Postsedation neuroexcitation is sometimes attributed to intravenous injection of the sedative-hypnotic drug propofol. The movements associated with these events have strongly suggested convulsive activity, but they rarely have been comprehensively evaluated. We present video recordings of 3 healthy young patients who underwent elective surgery under conscious sedation and emerged from sedation with transient but repetitive violent motor activity and impaired consciousness. These manifestations required considerable mobilization of multiple health care workers to protect the patient from inflicting harm. All patients received propofol, and all fully recovered without adverse sequelae. We postulate that these movements are propofol related. Importantly, we found no evidence of seizures clinically or electrographically.

Subarachnoid Hemorrhage and Spinal Subdural Hematoma Due to Acute CSF Hypotension.

BACKGROUND: Intracranial subarachnoid hemorrhage (SAH) and spinal subdural hematoma (SDH) are rare complications of spine surgery, thought to be precipitated by cerebrospinal fluid (CSF) hypotension in the setting of an intraoperative durotomy or postoperative CSF leak. Considerable clinical variability has been reported, requiring a high level of clinical suspicion in patients with a new, unexplained neurologic deficit after spine surgery. METHODS: Case report. RESULTS: An 84-year-old man developed symptomatic spinal stenosis with bilateral lower extremity pseudoclaudication. He underwent L3-5 laminectomy at an outside institution, complicated by a small, incidental, unrepairable intraoperative durotomy. On postoperative day 2, he became confused; and head CT demonstrated intracranial SAH with blood products along the superior cerebellum and bilateral posterior Sylvian fissures. He was transferred to our neurosciences ICU for routine SAH care, with improvement in encephalopathy over several days of supportive care. On postoperative day 10, the patient developed new bilateral lower extremity weakness; MRI of the lumbar spine demonstrated worsening acute spinal SDH above the laminectomy defect, from L4-T12. He was taken to the OR for decompression, at which time a complex 1.5-cm lumbar durotomy was identified and repaired primarily. CONCLUSIONS: We report the first case of simultaneous intracranial SAH and spinal SDH attributable to postoperative CSF hypotension in the setting of a known intraoperative durotomy. Although rare, each of these entities has the potential to precipitate a poor neurologic outcome, which may be mitigated by early recognition and treatment.

Mercy killing in neurology: The beginnings of neurology on screen (II).

The history of Neurocinema includes neuroethics, and this theme was first used in 2 films released in the 1940s in both Germany and the United States. Ich Klage An (I Accuse) is about "terminal" multiple sclerosis in a young woman and the decision to determine one's own fate. The protagonist anticipates becoming "deaf, blind, and idiotic" and asks her husband to administer a toxic drug dose, which he does. The film disturbingly suggests that the diagnosis of multiple sclerosis is tantamount to a death sentence. Ich Klage An (1941) played during the medical murders era ("Aktion T-4" program) but has few references to National Socialism, except for judges with Nazi emblems on their robes making a brief Nazi salute and a jury chamber with a bust of Hitler. Party leadership agreed that the film made a deep impression, but the intended effect on the viewing public is largely unknown. An Act of Murder (1948) involves another young woman with an inoperable brain tumor. When her condition worsens during a trip, her husband deliberately crashes the car, killing her but surviving himself. A subsequent trial finds that she died of an overdose rather than the crash. The trial judge dismisses the murder charge, but the film argues the morals of mercy killing. These films came out during the Nazi euthanasia program and founding of the Euthanasia Society of America in 1938. The choice of neurologic disease by these filmmakers and scriptwriters to defend euthanasia is remarkable.

"Dark Victory" (prognosis negative): The beginnings of neurology on screen.

In "Dark Victory," released in theaters in 1939, the diagnosis and management of a progressive brain tumor was a central part of the screenplay, and this film marked the beginnings of the depiction of neurologic disease in cinema. Bette Davis' cinematic portrayal of a young woman dying from a brain tumor is close to the reality of denial, bargaining, a hope for a cure, and final acceptance. "Dark Victory" includes part of a neurologic examination (funduscopy, testing of strength, testing of stereognosis, and tendon reflexes). The film also alludes to decisions on what to tell the patient (better say nothing) and shows an implausible clinical course (an abrupt peaceful ending). The film is unusual in depicting the presentation of a brain tumor, but the cinematic portrayal of the vicissitudes of living with a brain tumor is often close to reality.

Autoimmune Encephalitis in the ICU: Analysis of Phenotypes, Serologic Findings, and Outcomes.

BACKGROUND: To report the clinical and laboratory characteristics, clinical courses, and outcomes of Mayo Clinic, Rochester, MN, ICU-managed autoimmune encephalitis patients (January 1st 2003-December 31st 2012). METHODS: Based on medical record review, twenty-five patients were assigned to Group 1 (had ≥1 of classic autoimmune encephalitis-specific IgGs, n = 13) or Group 2 (had ≥3 other characteristics supporting autoimmunity, n = 12). RESULTS: Median admission age was 47 years (range 22-88); 17 were women. Initial symptoms included ≥1 of subacute confusion or cognitive decline, 13; seizures, 12; craniocervical pain, 5; and personality change, 4. Thirteen Group 1 patients were seropositive for ≥1 of VGKC-complex-IgG (6; including Lgi1-IgG in 2), NMDA-R-IgG (4), AMPA-R-IgG (1), ANNA-1 (1), Ma1/Ma2 antibody (1), and PCA-1 (1). Twelve Group 2 patients had ≥3 other findings supportive of an autoimmune diagnosis (median 4; range 3-5): ≥1 other antibody type detected, 9; an inflammatory CSF, 8; ≥1 coexisting autoimmune disease, 7; an immunotherapy response, 7; limbic encephalitic MRI changes, 5; a paraneoplastic cause, 4; and diagnostic neuropathological findings, 2. Among 11 patients ICU-managed for ≥4 days, neurological improvements were attributable to corticosteroids (5/7 treated), plasmapheresis (3/7), or rituximab (1/3). At last follow-up, 10 patients had died. Of the remaining 15 patients, 6 (24%) had mild or no disability, 3 (12%) had moderate cognitive problems, and 6 (24%) had dementia (1 was bed bound). Median modified Rankin score at last follow-up was 3 (range 0-6). CONCLUSIONS: Good outcomes may occur in ICU-managed autoimmune encephalitis patients. Clinical and testing characteristics are diverse. Comprehensive diagnostics should be pursued to facilitate timely treatment.

Acutely Trapped Ventricle: Clinical Significance and Benefit from Surgical Decompression.

BACKGROUND: Focal ventricular obstruction--trapped ventricle--results in cerebrospinal fluid accumulation, mass effect and possible clinical deterioration. There are no systematic studies on the benefit of surgical decompression in adults. METHODS: We reviewed patients admitted with acutely trapped ventricle on brain imaging to assess their prognosis and the effect of surgical intervention on 30-day mortality. RESULTS: Of the 392 patients with trapped ventricle, the most common causes were brain tumor (45%), intracerebral hemorrhage (ICH) (20%), and subdural hematoma (SDH) (14%). Lateral ventricle trapping accounted for 97% of cases. Two hundred and twenty-one patients (56%) received a surgical intervention for trapped ventricle or its causes; 126 (83%) were treated with craniotomy, 26 (17%) with craniectomy, 30 (14%) with external ventricular drain (EVD) alone, 23 (10%) with ventriculoperitoneal shunt alone, and 16 (7%) with endoscopic fenestration of the septum pellucidum. Surgical intervention was associated with mortality reduction from 95% (n = 54) to 48% (n = 11) in the ICH group, from 47% (n = 27) to 12% (n = 15) in the tumor group and from 90% (n = 18) to 20% (n = 7) in the SDH group (p < 0.001 for all comparisons). Univariate logistic analysis showed that surgical intervention and tumor etiology were associated with decreased mortality while age, ICH etiology, intraventricular hemorrhage, midline shift, and anticoagulation were associated with increased mortality. On multivariate logistic regression, surgical intervention remained associated with decreased mortality (p < 0.0001; OR 0.20, 95% CI 0.09-0.42). On subgroup analysis of the ICH cohort, surgical intervention was also associated with decreased mortality (p = 0.028). CONCLUSIONS: Neurosurgical intervention for decompression in patients with trapped ventricle can have a measurable beneficial effect on early mortality.

Transfusion-Related Acute Lung Injury After IVIG for Myasthenic Crisis.

BACKGROUND: A 26-year-old female with myasthenic crisis developed transfusion-related acute lung injury (TRALI) after she was treated with intravenous immunoglobulin. METHODS: Case report. RESULTS: Respiratory status markedly worsened with each intravenous immunoglobulin (IVIG) administration and progressing from a need to use bilevel positive airway pressure (BiPAP) to intubation. Pulmonary function tests improved during this episode. CONCLUSIONS: IVIG may cause TRALI and due to subtle clinical findings can be mistaken for neuromuscular respiratory failure.