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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.

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.

Objectives & methods Most of the studies since now show an impairment for facial displays of disgust recognition in Parkinson disease. A general impairment in disgust processing in patients with Parkinson disease might adversely affect their social interactions, given the relevance of this emotion for human relations. However, despite the importance of faces, disgust is also expressed through other format of visual stimuli such as sentences and visual images. The aim of our study was to explore disgust processing in a sample of patients affected by Parkinson disease, by means of various tests tackling not only facial recognition but also other format of visual stimuli through which disgust can be recognized. Results Our results confirm that patients are impaired in recognizing facial displays of disgust. Further analyses show that patients are also impaired and slower for other facial expressions, with the only exception of happiness. Notably however, patients with Parkinson disease processed visual images and sentences as controls. Conclusions Our findings show a dissociation within different formats of visual stimuli of disgust, suggesting that Parkinson disease is not characterized by a general compromising of disgust processing, as often suggested. The involvement of the basal ganglia-frontal cortex system might spare some cognitive components of emotional processing, related to memory and culture, at least for disgust.

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.

A group of 20 patients with probable Alzheimer disease (AD) and a control group were tested in a verb generation task, in a verb synonym task and several cognitive tests. Three types of verbs and novel verbs were presented in simple sentence frames, in two different conditions. In one condition participants were presented with the verb in the infinitive, providing information about the conjugation of the verb and the most likely type of past participle mapping required. The second was an ambiguous condition in which the suffix of the input verb did not provide any clue to the conjugation. The aim was to investigate if different types of verbs and input mapping affected patients' performance, and to what extent the deficit increased when the illness became more severe. Dependent measures were accuracy rates, rates of different morpho-phonological transformations and error type rates. Patients showed a more marked deficit in verb generation, when input was ambiguous. Correlation between the verb synonym test and accuracy in verb generation indicated that a deficit in lexical-semantic memory was partially responsible for impaired performance. Data suggest that patients maintained information about frequency distribution of different verb types and verb classes in each conjugation, but were impaired in operating complex phonological transformations.

– A systematic investigation of the cognitive functions of 22 patients affected with motor neuron disease (MND) compared to 36 controls matched for age and education was performed. The MND group showed cognitive performances slightly but significantly lower than the control group; 6 MND patients, however, had decidedly pathological values. Cognitive impairment was stereotyped and global, with sparing of memory. There was no significant difference between patients with isolated involvement of the lower motor neuron and those with associated pyramidal involvement. Our neuropsychological findings are in agreement with previous clinical, neuroradiological and pathological reports indicating extra-motor cerebral involvement in MND.

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.

The purpose of this study was to investigate the pattern of cognitive impairment in mild Alzheimer's disease (AD). We tested thirty patients (10 men and 20 women) with mini mental state examination (MMSE) scores between 20 and 24. The mental deterioration battery (MDB) was administered to all subjects. For each patient, the mean general score for the 4 verbal and the 4 non-verbal items were calculated, in order to verify the existence of a significant difference between them. In our sample the results showed that the difference between verbal and non-verbal items was not significant, i.e., the disease seems to affect both domains uniformly.

We examined by neuropsychological tests 41 patients who had presented attacks of transient global amnesia (TGA; 31 had single and 10 multiple episodes), comparing them with 41 matched normal controls. Patients with single attacks showed only two impaired memory tasks with respect to controls (immediate and long-term verbal memory), while patients with multiple attacks showed more impaired tasks in memory and visuoperceptual ability. These data confirm that TGA is a benign syndrome, but could leave a few subclinical memory deficits probably exacerbated by repeated attacks.

Three cirrhotic patients with chronic acquired hepatocerebral degeneration (CAHD) received neurologic, neuropsychologic and neuroimaging assessment before and after liver transplantation (LT). Before transplantation, neurologic dysfunction consisted in severe bradykinesia, dystonia, dyskinesia, ataxia and dysarthria. Cognitive impairment affected mainly attentional and executive domains. Brain MRI showed bilateral hyperintensities of the basal ganglia on T1-weighted images. After transplantation, motor manifestations promptly resolved. Cognitive testing showed a major improvement in two patients, whereas cognitive performances were slightly worsened in the third, reasonably due to the effects of a head injury before LT and a tacrolimus-related encephalopathy arising early after LT. MRI images 12 months later showed a slight reduction of the previously disclosed abnormalities in all three patients. None of them experienced recurrence of CAHD. Our observation reinforces the assumption that surgery is the best treatment option for CAHD and that severe neurological impairment in CAHD should not be considered a contraindication for LT.

We report the clinical findings and neuropsychological profiles of a sample of patients exhibiting a focal retrograde amnesia (FRA) seen consecutively during the period 1992–2007. The cohort comprised 13 patients, five males, with a mean age of 30 years (range 16–49). They were given a neurologic examination, psychiatric interview and formal neuropsychological examination (all but one) during the amnesic phase, underwent neuroimaging, and were followed up for six months to ten years. All presented with an acute amnesia characterized by an impaired recollection of memories predating the acute event, with spared or minimally and transiently affected anterograde memory, thus consistent with FRA. The events triggering FRA varied widely: mild to severe head injury, road accident without head injury, seizure, dissociative fugue, BDZ overdose, posttraumatic headache, syncope, migraine attack, acute distress. The neuropsychological hallmark of FRA was a selective or prominent impairment of autobiographical memory. The defect was often so severe as to cover most or all of the patients' lives and, in some cases, to erase the knowledge of their own identity. Conventional neuroimaging (brain CT and MRI) was unimpressive. Cerebral SPECT/PET disclosed unilateral frontal hypoperfusion in three (two left). All but one patient fully recovered, time of recovery ranging from three days to six months. FRA is a condition reflecting a block of memory function triggered by heterogeneous events, including both physical and psychic insults. Analogies shared with the more frequently encountered and better known condition of transient global amnesia suggests common pathogenetic mechanisms. A tentative nosographic classification of FRA is finally offered.

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.

CNS involvement is frequent in patients with chronic liver disease, resulting in overt or subclinical (“minimal”) encephalopathy. Occasionally, patients liver cirrhosis may develop a progressive encephalopathy known as chronic acquired hepatocerebral degeneration (CAHD), presenting with neuropsychiatric changes and movement disorders. In patients affected by CAHD cognitive dysfunction is the rule, but to date this aspect has not been systematically studied. Our aim was to characterize the neuropsychological profile of cognitive impairment associated with CAHD. Eight patients with CAHD received extensive neuropsychological assessment, far from episodes of acute liver decompensation. Their cognitive performances were compared with those of 8 patients with cirrhosis free from CAHD or overt hepatic encephalopathy (HE) and with those of 8 healthy controls matched for age, sex and educational level. Patients with CAHD revealed a significant impairment of visuo-spatial attention compared to healthy controls, and a lower performance on a single task of visual search and sequencing when compared to cirrhotics without CAHD. Our findings support the hypothesis of a linear decline in attentional performances of patients with chronic liver disease, starting from cognitively intact patients, moving toward patients with minimal HE, and finally progressing to those with overt HE and CAHD.

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.

We describe three patients exhibiting an acute reversible amnesia characterised by an impaired recollection of past events with preserved anterograde memory, thus consistent with a focal retrograde amnesia (FRA). This occurred after variable events: state of fugue, road accident, post-traumatic headache. Retrograde amnesia affected autobiographical memory so severely as to cover all of the patients' lives and to erase knowledge of their own identity. The retrieval of public events was variably affected, ranging from normality to severe impairment. No lesions were found on neuroimaging, and neurophysiological findings were unimpressive. FRA subsided in a few days, leaving a gap for the onset. The hypothesis of a psychogenic amnesia is considered, but overcoming the organic/psychogenic dichotomy the episodes appear as examples of "functional" memory inhibition, potentially triggered by different conditions, including events classifiable as psychic trauma. The clinical and neuropsychological traits of functional FRA are discussed. According to a current theory of autobiographical memory, the memory profile may be explained by a lost access to abstract autobiographical knowledge. Given some analogies with the more common transient global amnesia, a mechanism of spreading depression may also be hypothesised for functional FRA.

It is well-known that liver cirrhosis is frequently accompanied by a wide range of neuropsychiatric abnormalities, including general and specific cognitive impairment. The aim of this study was to investigate which cognitive functions are selectively compromised in Hepatic Encephalopathy (HE) and to clarify the relationship between clinically overt or nonovert HE and the different forms and degrees of decay in cognitive deficits. Twenty-two patients without overt HE and 12 patients who showed overt HE at the first level of severity, along with matched control subjects, were compared in several cognitive domains. The results showed significant differences in some measures of attention between patients with minimal HE (mHE) and patients with overt HE. There were also notable differences in verbal short-term memory between patients with mHE and healthy subjects. Thus, we can hypothesize that there is a linear diminution in short-term memory and attentional performance starting from healthy patients, moving toward patients with mHE, and finally progressing toward patients with the first grade of overt HE. There are two types of diminution that we noted: between patients with mHE and the overt form, the decline in the attentional domain was more evident, while between healthy subjects and mHE patients, short-term memory showed a more evident decline.

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.

A man experienced temporary amnesia for tasks which he was used to performing daily as they were the core of his working activity (bread making). No overt etiology was found. We hypothesized a disorder that is similar to transient global amnesia, but selectively affects procedural memory.

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.

A selective, temporally limited retrograde amnesia, confined to autobiographical memory, was the only sequela of a “minor” head trauma in two young men. The retrograde memory gap covered about one year of life before the trauma and persisted for several months, without any anterograde deficit or other cognitive disturbances. This unusual pattern of retrograde amnesia deserves close consideration and points to the dissociation of memory subsystems and mechanisms.

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 brief battery for mental deterioration assessment was obtained by Discriminant Analysis techniques from the Mental Deterioration Battery (MDB) (1) and yielded 98 % correct classifications in a sample of 60 subjects (30 pathological and 30 controls). This battery, named Brief Mental Deterioration Battery (BMDB), both quick and easy to administer, is composed of four tests: Rey's 15 Words Test, Immediate Visual Memory, Barrage, and Simple Analogies Test. MDB was administered to a further sample of 60 normal subjects and, by multivariate statistical techniques, a probabilistic definition of “normality” and consequently of “non-normality” was given. When applied to pathological and control groups, this probabilistic dichotomic classification yielded groups almost identical to the previous ones.

We systematically investigated the neuropsychological effects of controlled withdrawal of antiepileptic therapy with a battery of tests exploring intelligence, vigilance, attention, memory and sensori-motor performance. 16 patients without seizures for at least 2 years, 9 on therapy with phenobarbital (PB) and 7 with carbamazepine (CBZ), were examined 4 times over a period of 21 months. No significant correlation was found between drug levels and performance in the tests. The slight differences found between the PB and CBZ groups at full doses disappeared completely one year after withdrawal.

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.