Gayet-Wernicke Syndrome: The eye surgeon in a French neurologic eponym.

Gayet-Wernicke syndrome is an eponym mainly used in France. In this article, we revisit Charles Gayet's (1833-1904) speciality and his patient example that gave rise to the eponym. Charles Gayet attributed the anatomical lesions to inflammation. However, they were mainly due to hemorrhage, as Wernicke's term "polioencéphalite supérieure aiguë hémorragique" (polio-encephalitis superior haemorrhagica) explicitly indicates. The pathology of Gayet's case did not involve the mamillary bodies, colliculi, or cerebellum. Gayet did not mention abnormal memory functions, which are also cardinal signs of Wernicke-Korsakoff's disease. We argue that the Gayet-Wernicke eponym is not merited and that the more common international term "Wernicke-Korsakoff syndrome" should be used in France as elsewhere in the world.

Toxic-metabolic encephalopathy in adults: Critical discussion and pragmatical diagnostic approach.

Toxic-metabolic encephalopathy (TME) results from an acute cerebral dysfunction due to different metabolic disturbances including medications or illicit-drugs. It can lead to altered consciousness, going from delirium to coma, which may require intensive care and invasive mechanical ventilation. Even if it is a life-threatening condition, TME might have an excellent prognosis if its etiology is rapidly identified and treated adequately. This review summarizes the main etiologies, their differential diagnosis, and diagnostic strategy and management of TME with a critical discussion on the definition of TME.

Charcot in the ICU: functional tetraplegia after surgery.

Functional neurological disorder is a condition in which a patient has physical findings that are not compatible with anatomical boundaries, have no structural substrate and are not representable of an established disease. General anaesthesia and surgery have been previously reported as precipitating factors for functional disorders and mostly involve dissociative (non-epileptic) seizures. We report a patient with no psychiatric history or prior abnormal examination who developed sudden onset functional tetraplegia and sensory disturbances immediately after elective surgery, and who was subsequently discharged home several days later after nearly complete resolution of neurologic deficits. We highlight the features of this syndrome, including its unique postoperative presentation, unusual resolution and absence of any identifiable psycho-dynamic mechanism. We also introduce the tripod sign as a useful clinical tool in identifying functional tetraplegia.

Long-term mortality, disability and stroke recurrence in patients with basilar artery occlusion.

BACKGROUND AND PURPOSE: The long-term outcomes and stroke recurrence after basilar artery occlusion (BAO) are largely unknown. We aimed to assess these variables in a comparatively large series of consecutive patients. METHODS: Adults with acute BAO were retrospectively identified from 1976 to 2011. Post-discharge records were reviewed to assess for stroke recurrences, mortality and disability. Exploratory analysis of survival was carried out using Kaplan-Meier and log-rank tests. Factors associated with survival time were determined using Cox models. RESULTS: A total of 86 patients (34% female, median age 72 [interquartile range (IQR), 60-79] years) with a median National Institutes of Health Stroke Scale score of 11 (IQR, 6-27) were included. Twenty-nine patients (34%) died during the initial hospitalization. Median modified Rankin Scale (mRS) score at discharge among survivors was 4 (IQR, 2.5-5.5). At 1 and 5 years, 70% of survivors ad a mRS ≤3. Seventeen patients had recurrent strokes during the hospitalization and 12 patients had 19 recurrent strokes after discharge. The median survival time was 52 days (IQR, 6-1846). Older age per decade on admission [adjusted hazard ratios (aHR), 1.32; 95% confidence interval (CI), 1.05-1.66, P = 0.02] and a higher mRS at discharge (aHR, 4.48; 95% CI, 2.72-7.39, P < 0.0001) were associated with mortality. Patients who were not treated with any reperfusion therapy had a trend towards reduced mortality (aHR, 0.39; 95% CI, 0.14-1.08, P = 0.07). CONCLUSIONS: Survivors from BAO had severe short-term functional disability. Most deaths and stroke recurrences occurred within the first year following the initial event. The risk of death was higher in older and more disabled survivors. However, favorable long-term recovery was possible.

The scope of neurology of critical illness.

Critical illness increases the probability of a neurologic complication. There are many reasons to consult a neurologist in a critically ill patient and most often it is altered alertness with no intuitive plausible explanation. Other common clinical neurologic problems facing the intensive care specialist and consulting neurologist in everyday decisions are coma following prolonged cardiovascular surgery, newly perceived motor asymmetry, seizures or other abnormal movements, and generalized muscle weakness. Assessment of long-term neurologic prognosis is another frequent reason for consultation and often to seek additional information about the patient's critical condition by the attending intensivist. Generally speaking, consultations in medical or surgical ICU's may have a varying catalog of complexity and may involve close management of major acute brain injury. This chapter introduces the main principles and scope of this field. Being able to do these consults effectively-often urgent and at any hour of the day-requires a good knowledge of general intensive care and surgical procedures. An argument can be made to involve neurointensivists or neurohospitalists in these complicated consults.

Management of the comatose patient.

Coma has many causes but there are a few urgent ones in clinical practice. Management must start with establishing the cause and an attempt to reverse or attenuate some of the damage. This may include early neurosurgical intervention, efforts to reduce brain tissue shift and raised intracranial pressure, correction of markedly abnormal laboratory abnormalities, and administration of available antidotes. Supporting the patient's vital signs, susceptible to major fluctuations in a changing situation, remains the most crucial aspect of management. Management of the comatose patient is in an intensive care unit and neurointensivists are very often involved. This chapter summarizes the principles of caring for the comatose patient and everything a neurologist would need to know. The basic principles of neurologic assessment of the comatose patient have not changed, but better organization can be achieved by grouping comatose patients according to specific circumstances and findings on neuroimaging. Ongoing supportive care involves especially aggressive prevention of medical complications associated with mechanical ventilation and prolonged immobility. Waiting for recovery-and many do- is often all that is left. Neurorehabilitation of the comatose patient is underdeveloped and may not be effective. There are, as of yet, few proven options for neurostimulation in comatose patients.

Management of acute neuromuscular disorders.

Imminent neuromuscular respiratory failure is recognized by shortness of breath, restlessness, and tachycardia and is often followed by tachypnea, constantly interrupting speech, asynchronous breathing and sometimes paradoxical breathing and use of scalene and sternocleidomastoid muscles. Once a patient presents with such a constellation of signs, there are some difficult decisions to be made and include assessment of the severity of respiratory failure and in particular when to intubate. Failure of the patient to manage secretions as a result of oropharyngeal weakness rather than neuromuscular respiratory weakness may be another reason for acute intubation. Any patient with rapidly worsening weakness on presentation will need admission and observation in an intensive care unit. This chapter summarizes the pathophysiology of acute neuromuscular respiratory failure, its clinical recognition and respiratory management and outcome expectations.

Decompressive craniectomy in acute brain injury.

Decompressive surgery to reduce pressure under the skull varies from a burrhole, bone flap to removal of a large skull segment. Decompressive craniectomy is the removal of a large enough segment of skull to reduce refractory intracranial pressure and to maintain cerebral compliance for the purpose of preventing neurologic deterioration. Decompressive hemicraniectomy and bifrontal craniectomy are the most commonly performed procedures. Bifrontal craniectomy is most often utilized with generalized cerebral edema in the absence of a focal mass lesion and when there are bilateral frontal contusions. Decompressive hemicraniectomy is most commonly considered for malignant middle cerebral artery infarcts. The ethical predicament of deciding to go ahead with a major neurosurgical procedure with the purpose of avoiding brain death from displacement, but resulting in prolonged severe disability in many, are addressed. This chapter describes indications, surgical techniques, and complications. It reviews results of recent clinical trials and provides a reasonable assessment for practice.

The history of neurocritical care.

Critical care medicine came into sharp focus in the second part of the 20th century. The care of acutely ill neurologic patients in the USA may have originated in postoperative neurosurgical units, but for many years patients with neurocritical illness were admitted to intensive care units next to patients with general medical or surgical conditions. Neurologists may have had their first exposure to the complexity of neurocritical care during the poliomyelitis epidemics, but few were interested. Much later, the development of neurocritical care as a legitimate subspecialty was possible as a result of a new cadre of neurologists, with support by departments of neurosurgery and anesthesia, who appreciated their added knowledge and expertise in care of acute neurologic illness. Fellowship programs have matured in the US and training programs in certain European countries. Certification in the USA is possible through the American Academy of Neurology United Council of Neurologic Specialties. Most neurointensivists had a formal neurology training. This chapter is a brief analysis of the development of the specialty critical care neurology and how it gained strength, what it is to be a neurointensivist, what the future of care of these patients may hold, and what it takes for neurointensivists to stay exemplary. This chapter revisits some of the earlier known and previously unknown landmarks in the history of neurocritical care.

Devastating Delayed Leukoencephalopathy Associated with Bath Salt Inhalation.

BACKGROUND: "Bath salts" or synthetic cathinone toxicity remains a potentially deadly clinical condition. We report a delayed leukoencephalopathy with persistent minimally conscious state. METHODS: Case report. RESULTS: A 36-year-old man presents with delayed encephalopathy, dysautonomia, fulminant hepatic failure, and renal failure from severe rhabdomyolysis after consuming bath salts. MRI showed diffusion restriction in the splenium of the corpus callosum and subcortical white matter. CONCLUSIONS: The combination of acute leukoencephalopathy, rhabdomyolysis and fulminant hepatic failure may point to bath salt inhalation and should be known to neurointensivists.

Predictors of outcome in refractory status epilepticus.

OBJECTIVE: To further characterize the demographics, outcomes, and prognostic factors for refractory status epilepticus (RSE). DESIGN: Retrospective analysis of all the episodes of RSE treated between January 1, 1999, and August 30, 2011. SETTING: Neurointensive care unit within a tertiary referral center, Mayo Clinic, Rochester, Minnesota. PATIENTS: Refractory status epilepticus was defined as generalized convulsive or nonconvulsive status epilepticus (SE) that continued despite initial first- and second-line therapies. Exclusion criteria were aged younger than 18 years, anoxic/myoclonic SE, psychogenic SE, simple partial SE, and absence SE. MAIN OUTCOME MEASURES: Functional outcome was defined by modified Rankin scale (mRS) dichotomized into good (mRS, 0-3) and poor (mRS, 4-6). Functional decline was defined as a change in mRS greater than 1 from hospital admission to discharge. RESULTS: We identified 63 consecutive episodes of non-anoxic RSE in 54 patients. Anesthetic agents were used in 55 episodes (87.30%), and duration of drug-induced coma was (mean [SD]) 11.0 (17.9) days. In-hospital mortality was 31.75% (20 of 63 episodes). Poor functional outcome at discharge occurred in 48 of 63 episodes (76.19%). Hospital length of stay was (mean [SD]) 27.7 (37.3) days. Duration of drug-induced coma (P=.03), arrhythmias requiring intervention (P=.01), and pneumonia (P=.01) were associated with poor functional outcome. Prolonged mechanical ventilation was associated with mortality (P=.04). Seizure control without suppression-burst or isoelectric electroencephalogram predicted good functional recovery (P=.01). Age, history of epilepsy, previous SE, type of SE, and anesthetic drug used were not associated with functional outcome. CONCLUSIONS: Three-quarters of patients with RSE have a poor outcome. Achieving control of the SE without requiring prolonged drug-induced coma or severe electroencephalographic suppression portends better prognosis.

Second brain death examination may negatively affect organ donation.

BACKGROUND: Little is known about the impact of the requirement for a second brain death examination on organ donation. In New York State, 2 examinations 6 hours apart have been recommended by a Department of Health panel. METHODS: We reviewed data for 1,229 adult and 82 pediatric patients pronounced brain dead in 100 New York hospitals serviced by the New York Organ Donor Network from June 1, 2007, to December 31, 2009. We reviewed the time interval between the 2 clinical brain death examinations and correlated this brain death declaration interval to day of the week, hospital size, and organ donation. RESULTS: None of the patients declared brain dead were found to regain brainstem function upon repeat examination. The mean brain death declaration interval between the 2 examinations was 19.2 hours. A 26% reduction in brain death examination frequency was seen on weekends when compared to weekdays (p = 0.0018). The mean brain death interval was 19.9 hours for 0-750 bed hospitals compared to 16.0 hours for hospitals with more than 750 beds (p = 0.0015). Consent for organ donation decreased from 57% to 45% as the brain death declaration interval increased. Conversely, refusal of organ donation increased from 23% to 36% as the brain death interval increased. A total of 166 patients (12%) sustained a cardiac arrest between the 2 examinations or after the second examination. CONCLUSION: A single brain death examination to determine brain death for patients older than 1 year should suffice. In practice, observation time to a second neurologic examination was 3 times longer than the proposed guideline and associated with substantial intensive care unit costs and loss of viable organs.

Factors influencing time to death after withdrawal of life support in neurocritical patients.

OBJECTIVE: Improving our ability to predict the time of death after withdrawal of life-sustaining measures (WLSM) could have a significant impact on rates of organ donation after cardiac death and allocation of appropriate medical resources. We sought to determine which pre-WLSM clinical factors were associated with earlier time to death in patients with catastrophic neurologic disease. METHODS: We retrospectively analyzed all patients who underwent WLSM from 2002 to 2008 in a neurologic intensive care unit. Individuals who died within 60 minutes were compared to those who died beyond this time from the point of WLSM. Patients declared brain dead or not intubated and cases with insufficient data were excluded. Demographic, clinical, laboratory, and radiographic data were reviewed. Statistical analysis was based on multivariate logistic regression. RESULTS: A total of 149 comatose patients satisfied our inclusion criteria. A total of 75 patients had cardiac arrest in <60 minutes; 57% were male and 52% were older than 66 years. Ischemic stroke (30%) and intraparenchymal hemorrhage (52%) were the most frequent diagnoses. Absent corneal (odds ratio [OR] = 4.24, 95% confidence interval [CI] 1.57-11.5, p = 0.005) and cough reflexes (OR = 4.46, 95% CI 1.93-10.3, p = 0.0005), extensor or absent motor response (OR = 2.83, 95% CI 1.01-7.91, p = 0.048), and an oxygenation index greater than 4.2 (OR = 3.36, 95% CI 1.33-8.5, p = 0.011) were associated with earlier death. CONCLUSIONS: Specific neurologic signs and respiratory measurements are associated with earlier death after withdrawal of life-sustaining measures in the neurologic intensive care unit. This subset of comatose patients with irreversible neurologic injury may be suitable for organ donation after cardiac death protocols. These attributes need validation in a prospective data set.

Neurological complications following adult lung transplantation.

The full spectrum of neurologic complications and their impact on survival in lung recipients has not been reported. A retrospective cohort review of the Mayo Clinic Lung Transplant Registry (1988-2008) was performed to determine the range of neurologic complications in a cohort of adult lung recipients. Cox regression models were used to assess risk factors for neurological complications and death posttransplant. One hundred and twenty lung transplant recipients (53% women, median age at transplantation 53 years, range 21-73, median survival 4.8 years) were identified, of whom 95 had a neurological complication posttransplantation (median time to complication 0.8 years). Neurological complications were severe in 46 patients (requiring hospitalization or urgent care and evaluation) and were most often perioperative stroke or encephalopathy. Age predicted neurological complications of any type, whereas lung allocation score, bilateral lung transplantation, sex, underlying lung disease, elevated hemoglobin A1C, renal insufficiency and smoking history did not. Neurological complications of any severity (HR 4.3, 95% CI 2.2-8.6, p < 0.001) and high severity (HR 7.2, 95% CI 3.5-14.6, p < 0.001) were associated with increased risk of death. Neurological complications are common after lung transplantation, affecting 92% of recipients within 10 years. Severe neurologic complications are also common, affecting 53% of recipients within 10 years.

Status epilepticus as initial manifestation of posterior reversible encephalopathy syndrome.

We report 10 cases of status epilepticus (SE) in patients with posterior reversible encephalopathy syndrome (PRES). In all cases, SE brought PRES to medical attention. The majority of the cases had focal-onset complex partial SE. Complete resolution of SE was achieved after combined treatment of PRES and SE in all cases. SE in the setting of PRES carries a favorable prognosis but requires timely recognition and treatment of the course of PRES.