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Chronic acquired hepatocerebral degeneration (CAHD) is a rare neurologic syndrome occurring in patients with chronic liver disease, resulting in the combination of movement disorders and cognitive\behavioral changes. Its pathogenesis is debated and the symptoms are poorly responsive to medical therapy. Meige's syndrome is a form of cranial dystonia, considered an idiopathic form of adult onset dystonia. We report a 60-year-old man with HCV-related liver cirrhosis and hepatocarcinoma who developed Meige's syndrome associated with cognitive and behavioral manifestations, unrelated to acute metabolic derangement. CAHD was diagnosed. Liver transplantation reversed the clinical picture and MR abnormalities, reinforcing the idea that CAHD is a potentially reversible syndrome, which may be healed by liver transplantation and should not be considered a contraindication for this operation.

Chimeric antigen receptor (CAR) T-cell therapy is an emerging highly effective treatment for refractory haematological malignancies. Unfortunately, its therapeutic benefit may be hampered by treatment-related toxicities, including neurotoxicity. Early aggressive treatment is paramount to prevent neurological sequelae, yet it potentially interferes with the anti-cancer action of CAR T-cells. We describe four CAR T-cells infused patients who presented with reiterative writing behaviours, namely paligraphia, as an early manifestation of neurotoxicity, and eventually developed frontal predominant encephalopathy (one mild, three severe). Paligraphia may represent an early, specific, and easily detectable clinical finding of CAR T-cell therapy-related neurotoxicity, potentially informing its management.

Background: Rapid management can reduce the short stroke risk after transient ischaemic attack (TIA), but the long-term effect is still little known. We evaluated 3-year vascular outcomes in patients with TIA after urgent care. Methods: We prospectively enrolled all consecutive patients with TIA diagnosed by a vascular neurologist and referred to our emergency department (ED). Expedited assessment and best secondary prevention was within 24 h. Endpoints were stroke within 90 days, and stroke, myocardial infarction, and vascular death at 12, 24 and 36 months. Results: Between August 2010 and July 2013, we evaluated 686 patients with suspected TIA; 433 (63%) patients had confirmed TIA. Stroke at 90 days was 2.07% (95% confidence interval (CI), 1.1-3.9) compared with the ABCD2-predicted risk of 9.1%. The long-term stroke risk was 2.6% (95% CI, 1.1-4.2), 3.7% (95% CI, 1.6-5.9) and 4.4% (95% CI, 1.9-6.8) at 12, 24 and 36 months, respectively. The composite outcome of stroke, myocardial infarction, and vascular death was 3.5% (95% CI, 1.7-5.1), 4.9% (95% CI, 2.5-7.4), and 5.6% (95% CI, 2.8-8.3) at 12, 24, and 36 months, respectively. Conclusions: TIA expedited management driven by vascular neurologists was associated with a marked reduction in the expected early stroke risk and low long-term risk of stroke and other vascular events.

Foreign accent syndrome (FAS) is a speech disorder that leads listeners to perceive the patient as having a foreign accent. The cardinal feature of FAS is prosody change, with variation in accent, intonation, and rhythm. Less frequently, phonetic changes with normal prosody may be detected. The composite features of patients with FAS are not characteristic of dysarthria or aphasia and partially resemble patients who have speech apraxia. Patients with FAS are often unfamiliar with the foreign language and often seem unaware of the disturbance, even though their ability to discriminate foreign accents is spared.

BACKGROUND The long-term effect of liver transplantation (LT) on cognitive functions and the complete reversibility of minimal hepatic encephalopathy are poorly documented. Much evidence indicates that spatial attention improves starting from the immediate period after LT. However, at least in the first 2 years, some cognitive defects seem to persist to some degree, especially for supramodal nonverbal cognitive functions. The aim of this study is to investigate (i) whether the improvements observed in the perioperative period fluctuate or remain stable 10 years after LT and (ii) whether the functions that have been found defective also improve. METHODS We called patients previously included in a prospective study (Mattarozzi et al., Arch Neurol 2004; 61: 242) for a further neuropsychological evaluation. We compared the cognitive evaluation after 7 to 10 years with previous data gathered 6 and 18 months after LT. RESULTS The improvements obtained in the first 2 years after transplantation remain stable during the 7 to 10 years thereafter, especially for visuospatial attention, F(12,96) 1.70; P=0.04 and selective attention, F(6,66) 3.51; P=0.005. Furthermore, these findings also seem to suggest an improvement in supramodal cognitive functions, such as spatial planning intelligence, measured by the Elithorn Maze Test, F(3,33) 7.42; P=0.002. Verbal short-term memory, F(3,33) 3.69; P=0.038, and visuospatial short-term memory, F(6,64) 2.97; P=0.013, show a more fluctuating trend over time. CONCLUSIONS Despite the risk of surgery, the neurotoxicity of immunosuppression therapy, and the effects of aging and related comorbidities, our data indicate that LT is able to significantly improve patients' cognitive functions in the long term.

Low-grade or minimal hepatic encephalopathy (MHE) is characterised by relatively mild neurocognitive impairments, and occurs in a substantial percentage of patients with liver disease. The presence of MHE is associated with a significant compromise of quality of life, is predictive of the onset of overt hepatic encephalopathy and is associated with a poorer prognosis for outcome. Early identification and treatment of MHE can improve quality of life and may prevent the onset of overt encephalopathy, but to date, there has been little agreement regarding the optimum method for detecting MHE. The International Society on Hepatic Encephalopathy and Nitrogen Metabolism convened a group of experts for the purpose of reviewing available data and making recommendations for a standardised approach for neuropsychological assessment of patients with liver disease who are at risk of MHE. Specific recommendations are presented, along with a proposed methodology for further refining these assessment procedures through prospective research.

Orthotopic liver transplantation (OLT) represents the sole etiological treatment for Wilson's disease (WD), but its efficacy in resolving the neurological symptoms is still a matter of debate. We present the case of a young male affected with WD with hepatic and neurological involvement, who underwent OLT for subacute liver failure. Clinical, neuropsychological and neuroradiological assessment were performed before and after OLT. OLT had no effect on neurological symptoms, suggesting that the basal ganglia damage may be irreversible. Nineteen months after OLT, the patient went on to develop new permanent extrapyramidal symptoms. We postulate a sequela of an early post-operative central pontine and extrapontine myelinolysis.

Background: Brain edema and increased intracranial pressure worsen prognosis in patients with end-stage chronic cirrhosis. Objective: To use diffusion-weighted imaging (DWI) to quantify water apparent diffusion coefficient (ADC) in different brain regions of patients with chronic liver failure with or without hepatic encephalopathy. Methods: The authors studied 14 patients with viral liver cirrhosis and 12 sex- and age-matched healthy volunteers. Seven patients had no clinical evidence of hepatic encephalopathy; six had grade I hepatic encephalopathy; and one had grade II hepatic encephalopathy. Brain DWI was obtained using a single-shot echo-planar imaging sequence, and four gradient strengths ( b values = 0, 300, 600, and 900 s/mm 2 ) were applied to calculate the average diffusivity maps. Results: Mean ADC values in the brains of patients with cirrhosis were significantly increased in all selected regions of interest (caudate, putamen, and pallidus nuclei; occipital, parietal, and frontal lobe white matter) except in the thalamus. Venous ammonia was linearly related to ADC values in deep gray and white matter regions of interest. Conclusions: Brain water apparent diffusion coefficient is increased in patients with chronic liver disease and may be useful in monitoring patients with hepatic encephalopathy.

Liver transplantation (LT) is the best treatment for end-stage liver diseases but it entails a high incidence of neuropsychiatric complications. These may be related to the operation or occur postoperatively, usually within the first month. The occurrence of neurological problems after LT increases the risk of mortality. The etiology of such complications is various, often multifactorial, immunosuppression being one of the most important causes. Immunosuppressive drugs may cause a wide spectrum of neuropsychiatric complications--mainly affecting the CNS--ranging from mild to severe disorders. A survey of the most frequent disorders is presented. In the management of liver-transplanted patients, the awareness of potential neurological and psychiatric problems is crucial for patients' survival, since it assists clinicians in prevention, prompt diagnosis, and treatment.

A right brain-damaged patient (CC) with visual neglect was evaluated for the presence and severity of anosognosic disorders, using a test described previously (Berti, Ladavas, & Della Corte, 1996). The patient was repeatedly questioned, on different occasions, about her motor deficits in an attempt to investigate and confront her denial behaviour. The patient showed a severe anosognosia for motor impairment in verbal report, without emisomatoagnosia. However, ''tacit'' knowledge of her physical condition was apparent in most conversations with the patient. Moreover, when asked to score her motor ability, she showed a dissociation between anosognosia on the verbal report and on the self-evaluation test. We conclude that investigations of anosognosia should take account of the patient's personal report and that this is crucial both for disclosing implicit mental content and for interpreting the underlying structure of conscious mental processes.

Intestinal transplantation has become a life-saving therapy in patients with irreversible loss of intestinal function and complications of total parenteral nutrition, but it entails a high incidence of neurologic complications, which occur more frequently than in other solid organ transplantations. Neurologic complications may be surgery-related or occur postoperatively, often within the first month. The etiology is various, often multifactorial, immunosuppression being one of the leading causes as intestine transplant recipients usually need potent immunosuppressive regimens. Neurologic problems after intestinal transplantation may present with a wide clinical spectrum, mainly mild to severe central nervous system disorders. Awareness of these complications is crucial for patients’ survival, since it will assist clinicians in prevention, prompt diagnosis, and treatment. This survey tackles the most common neurologic disorders and presents practice points of management which can help in the daily clinical care of intestinal transplant recipients.

National Library of Medicine from January 2005 to June 2009, the Cochrane Library, existing guidelines (National Clinical Clearinghouse, Scottish Intercollegiate Guidelines Network, National Institute of Clinical Excellence) and textbooks. Data collection and analysis of evidence was performed independently by each participant according to the above assignment. On the basis of the single reports, A.S. produced a fi rst draft of the updated guidelines, which was then submitted several times for the approval of all the members until any discrepancies on each topic were solved and a consensus was reached.

We report an unusual case of sudden isolated transient amnesia triggered by acute marijuana use. The memory disorder, apart from the long duration, had the characteristics of a transient global amnesia-like episode. Acute marijuana intoxication can affect memory more globally and severely than previously reported.

Neurological impairment after orthotopic liver transplantation (OLT) is common and represents a major source of morbidity and mortality. The diagnosis and management of neurological problems occurring after OLT are difficult and evidence-based guidelines for this task are currently lacking. A Task Force was set up under the auspices of the European Federation of Neurological Societies to devise guidelines to prevent and manage neurological problems in OLT. We selected six major neurological problems and approached them combining an evidence-based scientific literature analysis with a search of consensus by means of a Delphi process. Search results were translated into a series of recommendations constituting a basis for better care of patients with neurological complications after OLT.

Post-transplant headache is a recognized complication of organ transplantation. Its treatment can sometimes be problematic given the status of the patient and the risk of drug interference. Six organ-transplanted patients (5 liver and 1 heart) experiencing post-transplant headache were successfully treated with riboflavin 200 mg daily. The drug proved safe and well tolerated and its properties are unlikely to produce clinically relevant drug interactions in the transplant recipient. Awaiting specific trials, riboflavin is proposed as an option for the preventive treatment of post-transplant headache.

Brain edema and increased intracranial pressure, well recognised major complications of fulminant hepatic failure,1 have recently been documented in patients with end-stage cirrhosis.2 Neuroradiological evidence of cerebral edema in patients with chronic liver disease is associated with a poor prognosis. Medical treatments for brain edema in patients with cirrhosis have limited efficacy and, in general, liver transplantation is the only intervention which may prevent a fatal outcome.2 During the last 10 years several in vivo studies using magnetic resonance spectroscopy (MRS) have contributed to progress in understanding the neurochemical mechanisms responsible for hepatic encephalopathy (HE) and brain edema in cirrhotic patients.3, 4, 5, 6, 7, 8, 9, 10, 11 Proton (1H-) and carbon (13C-) MRS studies of the brain of cirrhotic patients with overt and minimal HE have typically shown an increase in glutamine,3,5, 6, 7, 8,12 a decrease in glutamate,6,8, 9, 10 and a decrease in the cerebral osmolyte myoinositol.4, 5, 6, 7 An increase in brain glutamine, the result of ammonia detoxification in astrocytes, has been implicated as the key metabolic change determining cell swelling and increased brain water in patients with HE.13,14 Studies on rats have shown that inhibition of glutamine synthesis with methionine-sulfoximine prevents the development of ammonia-induced brain edema,15 decreases ammonia-induced astrocyte swelling,16 and reduces brain edema after portacaval anastomosis and ammonia infusion.17 The impaired cell volume homeostasis and brain edema in patients with HE may be the result of intracellular accumulation of osmotically active glutamine associated with a progressive depletion of myo-inositol and other osmolytes.

Twelve consecutive patients having previously experienced one or more episodes of isolated transient topographical amnesia (TTA) underwent neuropsychological examination 6-12 months after the last episode to detect signs of global cognitive decline and to assess, in particular, cognitive functions deemed critical for topographical orientation. The test scores were compared with those of 12 normal subjects matched for age, sex, educational level and place of residence. Both patients and controls showed normal performances in all tasks. The comparison between the two groups revealed a significantly lower performance of the patient group in a task of geographical orientation, namely 'Map of Italy'. We conclude that isolated episodes of TTA in the elderly per se do not represent a sign of mental deterioration, but can be associated with frailty on survey components of spatial orientation. It remains to be explained whether this feature is a pre-existing condition favouring the occurrence of the episodes of topographical amnesia or represents a sequela.

Background Liver transplantation (LT) is the sole resolutive therapy for Wilson disease (WD) and is the treatment of choice for patients with WD who have fulminant hepatic failure or end-stage cirrhosis. Although its role in managing the neurological manifestations of WD is not yet conclusive, LT has recently been advocated as a therapy for neurologically affected patients with WD with stable liver function. Objective To evaluate the effect of LT on the neurological manifestations of WD. Observation A 44-year-old man with WD with cirrhosis and neurological symptoms (motor dysfunction and cognitive impairment) experienced a dramatic improvement in motor function early after LT, as well as normalization of copper balance and the disappearance of Kayser-Fleischer rings. Abnormalities seen on magnetic resonance imaging scans were reversed 18 months after LT. Cognitive testing 2 years after LT showed a moderate global improvement. Conclusions In this case, LT healed the neurological manifestations of WD. To date, this favorable result has been seen in almost 80% of cases. However, the decision to perform LT in patients with WD solely on the basis of neurological impairment must be considered experimental.

We describe a case of acute generalized dystonia due to bilateral damage to the basal ganglia, mainly affecting the globi pallidi. The lesion was probably related to a hypoxic condition following a heroin injection. Therapy with pimozide almost completely controlled the dystonia.

We examined 199 consecutive patients who underwent 220 liver transplantations, to define the type, frequency and aetiology of posttransplant neurological complications and their prognostic value. We found neurological complications in 63 patients (32%), mostly involving the central nervous system. The most frequent complications were mental status changes ranging from delirium to coma and seizures. The aetiology was multifactorial, cyclosporin A neurotoxicity being the main cause. Patients with neurological complications had a higher mortality rate than those without. In our series, neurological complications represented a major medical problem with increased morbidity and mortality.

Multiple sclerosis (MS) usually starts in young adulthood. However, the disease may appear late or very late in life. We report 6 cases with onset after the age of 59 years and review the literature. As in early onset disease, the diagnosis is mainly clinical. Laboratory findings and paraclinical evidence may support the diagnosis of clinical data are not sufficient. In elderly patients clinical history and laboratory data should be thoroughly appraised to exclude conditions more common in old age such as vascular diseases.

A patient with Parkinson’s disease developed permanent palilalia after bilateral stereotaxic thalamotomy.

Diffuse leptomeningeal infiltration by neoplastic cells is a well-recognized manifestation of malignant tumors. The usual clinical presentation is subacute or chronic meningitis with multifocal cranial and spinal nerve root symptoms and a variable degree of encephalopathy. We describe two patients with carcinomatous and leukemic meningitis mimicking Miller-Fisher syndrome. 1 Report of Cases. —Case 1. —A 73-year-old woman underwent a gastrectomy for cancer in July 1993. Six months later, she was admitted for diplopia, unsteadiness, and intermittent occipital headache that had started 3 days before. Neurologic examination disclosed bilateral abducens nerve paralysis, severe ataxia, and global areflexia. An electroencephalogram and a computed tomographic scan of the brain were normal. Cerebrospinal fluid (CSF) examination showed elevated proteins (0.66 g/L) with 1 cell per milliliter, and a low glucose level (0.34 g/L). Cytologic examination of the CSF disclosed adenocarcinomatous cells. Despite chemotherapy, the patient's condition became worse with rapid deterioration and death 20

A series of 92 patients with lacunar infarcts identified by CT brain scan are described. Risk factors for cerebrovascular disease, clinical aspects and outcome are analyzed. Our cases show unusual clinical syndromes in a remarkable number of subjects, indicating the usefulness of performing a CT brain scan.

The incidence of stroke among inpatients is not known. The aim of our study was to investigate the incidence of stroke not preceded by evident iatrogenic factors such as surgical or medical procedures in a cohort of inpatients in a large Italian general hospital. From January 1, 1992, to December 31, 1992, we evaluated patients referred to our neurology department with a suspected diagnosis of stroke that occurred during hospitalization. Patients presenting with stroke as a complication of iatrogenic causes were excluded. We calculated the incidence rate of first-ever stroke in our cohort (crude and among patients aged older than 50 years), thereafter adjusting these rates for age to the general population of the city district of Bologna (Italy). In 1992, 22 inpatients had a first-ever stroke with no evidence of iatrogenic factors. The crude stroke incidence rate was 11.08/1000 per year (95% confidence interval, 6.95 to 16.73). The age-adjusted rate was 5.46 (95% confidence interval 3.42 to 8.24). The incidence rate of first-ever stroke among hospitalized patients is higher than those reported in community-based studies. Higher frequency of coronary artery disease among our patients could explain our findings. Further studies are needed to identify possible predisposing factors (individual or environmental) for stroke among inpatients.

To the Editor. —Thrombotic thrombocytopenic purpura (TTP) is a potential but infrequent cause of cerebral ischemia in young adults. 1 In a recent survey of 144 young patients with nonhemorrhagic cerebral infarction, 2 no cases of TTP are reported. Computed tomographic (CT) evidence of cerebrovascular damage in TTP is extremely rare. 3 Report of a Case. —We recently admitted a 27-year-old woman because of recurrent attacks of right-sided central facial palsy (five in 36 hours), accompanied by slight fever. When she was not experiencing the attacks, her neurologic examination was normal. An electroencephalogram showed an excess of theta activity in the left frontotemporal regions. An electrocardiogram was normal. Blood parameters revealed severe hemolytic anemia (hemoglobin concentration, 57 g/L [5.7 g/dL], corrected reticulocyte count, 0.15 [15%]), severe thrombocytopenia (platelet count, 20 X 10 6 /L [20 000/mm 3 ]), and mild disseminated intravascular coagulation (DIC) (fibrinogen level, 1.25 g/L [125 mg/dL]