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.

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.

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.

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.

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.

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.

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.

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.

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.

Hydrocephalus is a determinant of early mortality in putaminal hemorrhage.

BACKGROUND AND PURPOSE: Previous studies have shown that the volume of intracerebral hemorrhage and Glasgow Coma Score (GCS) on admission are powerful predictors of 30-day mortality. However, the significance of hydrocephalus associated with deep cerebral hemorrhage has not been studied extensively. The purpose of this study was to determine the prognostic indicators of 30-day mortality in patients with deep cerebral hemorrhage. METHODS: We studied 100 consecutive patients with deep cerebral hemorrhage between 1994 and 1998. Deep cerebral hemorrhage was divided into 2 groups: putaminal hemorrhage (lateral group) and thalamic and caudate hemorrhage (medial group). Univariate and multivariate logistic regression analyses were performed to determine independent prognostic indicators of 30-day mortality. RESULTS: Hydrocephalus was present in 40 of the 100 patients. The 30-day mortality was 29%, and hydrocephalus was present in 76% of those who died. Multivariate analyses showed 2 independent prognostic indicators of 30-day mortality for putaminal hemorrhage: GCS

Neuroimaging in deteriorating patients with cerebellar infarcts and mass effect.

BACKGROUND AND PURPOSE: The decision to proceed with surgery in cerebellar infarct with mass effect (CIMASS) in deteriorating patients is based on clinical features. The potential role of neuroimaging in predicting deterioration has not been systematically studied. In this study we determine the role of neuroimaging in predicting deterioration in CIMASS. METHODS: -We retrospectively reviewed the clinical and neuroimaging features in 90 patients with cerebellar infarcts. We selected for detailed analysis CIMASS in 35 patients. RESULTS: Eighteen patients remained stable and 17 deteriorated. Of these 17 patients, 8 were treated conservatively and 9 had surgery. Radiological features indicative of progression were more common in deteriorating patients compared with stable patients: fourth ventricular shift (82.3% versus 50%, P:=0.075, OR=4. 67), hydrocephalus (76.5% versus 11.1%, P:=0.0001, OR=26), brain stem deformity (47% versus 5.6%, P:=0.0065, OR=15.1), and basal cistern compression (35.3% versus 0%, P:=0.0076, OR=20.91). Differences in upward displacement of the aqueduct and pontomesencephalic junction from Twining's line, tonsillar descent on sagittal MRI, and infarct volumes between stable and deteriorating patients were not statistically significant. CONCLUSIONS: Hydrocephalus, brain stem deformity, and basal cistern compression may herald deterioration in CIMASS. Admission to a neurological-neurosurgical intensive care unit and consideration of preemptive surgery are warranted in these patients. Vertical displacement of tonsils or aqueduct, demonstrated by MR imaging, did not predict deterioration.

The role of hypotension in septic encephalopathy following surgical procedures.

To study the risk factors for septic encephalopathy, we reviewed a consecutive series of 84 patients with septic syndrome and multiple-organ failure. Septic encephalopathy developed in 14 of these patients. Univariate analysis revealed that severe hypotension was significantly associated with the development of septic encephalopathy. In stepwise multiple logistic regression analysis, no other variable made a significant contribution in the presence of severe cardiovascular failure. Septic encephalopathy may be related to ischemic damage rather than to metabolic causes.

Blood pressure fluctuations in the dysautonomia of Guillain-Barré syndrome.

A 76-year-old man with severe Guillain-Barré syndrome had extremes of hypotension alternating with hypertension. His blood pressure paralleled both systemic vascular resistance and cardiac output. Heart rate, rather than stroke volume, was the major determinant of cardiac output over a wide range of blood pressures. It was at times invariant for several hours and was unresponsive to carotid massage or respiratory cycles, but slowed slightly with each episode of hypotension. Trend monitoring indicated that hypotension preceded reductions in pulmonary artery diastolic pressure. These findings suggest that hypotension resulted from a vasodepressor response with a vagotomized heart and that hypertension was the result of increased sympathetic activity. Both extremes were caused by parallel changes in vascular resistance and heart rate. Dysfunction of baroreflex buffering may have accounted for the rapid swings in pressure.

Acute relapsing Guillain-Barré syndrome after long asymptomatic intervals.

Five patients who recovered from an initial episode of Guillain-Barré syndrome had acute relapses 4, 10, 15, 17, and 36 years later, respectively. Two patients had multiple subsequent relapses. The antecedent illnesses, distribution of weakness, and clinical courses of each relapse were similar for each patient, except that relapses in three patients were briefer than the initial episode. One patient had asymptomatic sarcoidosis. Pharyngeal, oculomotor, and diaphragmatic weakness requiring a ventilator were common. Complete recovery or mild residual deficits, return of reflexes, normal cerebrospinal fluid protein at the onset of recurrent episodes, and normal or virtually normal nerve conduction velocities at various times distinguished these patients from those with more typical chronic relapsing inflammatory polyneuropathy.

Safety of discontinuation of anticoagulation in patients with intracranial hemorrhage at high thromboembolic risk.

BACKGROUND: Limited data are available to guide the management of anticoagulation in patients with intracranial hemorrhage (ICH) at high thromboembolic risk. OBJECTIVE: To review the management of anticoagulation in patients with ICH at high thromboembolic risk. PATIENTS AND METHODS: We reviewed the management of anticoagulation in 141 patients who have a high risk of ischemic stroke and have ICH while taking warfarin. The 30-day risk of ischemic stroke while not taking anticoagulation treatment was determined using Kaplan-Meier survival estimates. RESULTS: The indications for anticoagulation were a prosthetic heart valve (52 patients [group 1]), atrial fibrillation and cardioembolic stroke (53 patients [group 2]), and a recurrent transient ischemic attack or an ischemic stroke (36 patients [group 3]). A prior ischemic stroke occurred in 14 (27%) of group 1 patients and in 23 (43%) of group 2 patients. Death occurred in 43% of the 141 patients. The median time not taking warfarin in this cohort was 10 days. Three patients had an ischemic stroke within 30 days of warfarin therapy discontinuation. Using Kaplan-Meier survival estimates, the probability of having an ischemic stroke at 30 days following warfarin therapy cessation in groups 1, 2, and 3 was 2.9% (95% confidence interval, 0%-8.0%), 2.6% (95% confidence interval, 0%-7.6%), and 4.8% (95% confidence interval, 0%-13.6%), respectively. In the 35 patients who had warfarin therapy restarted, none had recurrence of ICH during the same hospitalization. CONCLUSIONS: Discontinuation of warfarin therapy for 1 to 2 weeks has a comparatively low probability of embolic events in patients at high embolic risk. This should be taken into consideration when deciding whether to continue or discontinue anticoagulation in these patients at high embolic risk. Early recurrence of ICH is exceedingly uncommon.

Warning headache in aneurysmal subarachnoid hemorrhage. A case-control study.

Thirty consecutive patients with aneurysmal subarachnoid hemorrhage (SAH), 20 patients with ischemic stroke, and 100 controls were extensively interviewed about previous episodes of sudden headache, according to a standard pro forma. Thirteen patients with SAH (43%) had a history of a forewarning headache, compared with only one of the patients with ischemic stroke and none of the controls. The interval from the warning headache to the admission rupture was between one week and two months in all patients but one with SAH. Only half of the 13 patients with a warning headache consulted their physician. The outcome was slightly worse in patients with a warning headache, but the differences did not reach statistical significance. These data emphasize the frequent occurrence of warning headaches in SAH. Measures to increase the recognition of sudden headaches should be considered.