Chronic acquired hepatocerebral degeneration: effects of liver transplantation on neurological manifestations.

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.

EFNS guidelines on management of neurological problems in liver transplantation.

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.

Shared cortical anatomy for motor awareness and motor control.

In everyday life, the successful monitoring of behavior requires continuous updating of the effectiveness of motor acts; one crucial step is becoming aware of the movements one is performing. We studied the anatomical distribution of lesions in right-brain-damaged hemiplegic patients, who obstinately denied their motor impairment, claiming that they could move their paralyzed limbs. Denial was associated with lesions in areas related to the programming of motor acts, particularly Brodmann's premotor areas 6 and 44, motor area 4, and the somatosensory cortex. This association suggests that monitoring systems may be implemented within the same cortical network that is responsible for the primary function that has to be monitored.

Verbal versus non-verbal performances in mild Alzheimer's disease.

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.

Diffusion MRI shows increased water apparent diffusion coefficient in the brains of cirrhotics.

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.

Cognitive functions after transient topographical amnesia.

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.

Neuropsychiatric complications of liver transplantation.

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.

Effect of liver transplantation on neurological manifestations in Wilson disease.

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.

Transient amnesia triggered by acute marijuana intoxication.

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.

Transient procedural amnesia.

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.

Neurological complications of liver transplantation.

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.

Acute generalized dystonia due to a bilateral lesion of basal ganglia mainly affecting the nuclei pallidi.

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.

No neurological improvement after liver transplantation for Wilson's disease.

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.