Andrea Stracciari

Neuropsychological assessment of hepatic encephalopathy: ISHEN practice guidelines

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.

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.

Multiple Sclerosis with Very Late Onset: Report of Six Cases and Review of the Literature

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.

Distinguishing Between Clinical and Minimal Hepatic Encephalopathy on the Basis of Specific Cognitive Impairment

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.

Cognitive functioning in chronic acquired hepatocerebral degeneration

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.

Development of Palilalia after Stereotaxic Thalamotomy in Parkinson’s Disease

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

Short- and Long-Term Stroke Risk after Urgent Management of Transient Ischaemic Attack: The Bologna TIA Clinical Pathway

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.

Transient global amnesia and transient topographical amnesia: an observation favoring the hypothesis of a common pathogenesis.

Sirs: I have previously focused attention on episodes of transient topographical amnesia (TTA) occurring in healthy elderly women, in the absence of overt brain disease or damage [5–7]. A TTA attack is characterized by sudden failure to find the way, in spite of a spared recognition of the environment. The nature of TTA is not clear; the only reasonable conjecture in most cases is that it reflects a transient dysfunction of unknown origin of the right occipitotemporal region. Similarities with the more common transient global amnesia (TGA) have been noted [5, 6]. Here I describe a patient who experienced two episodes of TGA, one of which ended in a typical TTA attack. This observation supports the idea of a common pathogenetic mechanism underlying both TGA and TTA. A 56-year-old right-handed healthy woman was seen for acute amnesia. On 14 March 1996, while swimming in a swimming pool with two friends, she suddenly lost her memory. She got out of the water, appeared astonished, asking the friends about why they had decided to go to the swimming pool (it was their regular activity every week). She repeated the same questions, showing a blank for the previous months. She dried herself, showing no problems in reaching the dressing room, in using towels and hair dryer, or dressing herself. She remained 90 minutes in the swimming pool area, during which the amnesic disturbance gradually subsided. Then, she decided to go home, driving her car by herself. She performed all the procedures to turn on the car engine and started. While driving, she suddenly lost her way, even though the route was familiar to her. After twenty minutes of driving around close to the swimming pool building and asking for help from passers by, she returned to the swimming pool and was taken to hospital. I saw the patient the next day. She appeared alert and fully oriented. Her medical history was unremarkable. In particular, no history of migraine, seizures, cerebrovascular disease or psychiatric disorders was reported. General and neurological examination were normal. Concerning the previous day, the patient had a blank from the moment she had begun to swim, until when she was outside in the car park (a period of about two hours), ready to get into her car to go home. She remembered having tried to drive along the usual route, but without being able to find her way, even though she recognized streets and buildings perfectly. Blood parameters, including thyroid function, and ECG proved normal. Search for alcohol and drugs was negative. EEG disclosed isolated theta activity on the temporal regions. Brain CT and ultrasound Doppler echotomography proved normal. A neuropsychological examination gave normal findings. Two months later cerebral SPECT was normal. On 6 February 1997, the patient experienced a 40 minute long antero-retrograde amnesia, again triggered by swimming. Neurological examination, EEG and brain MRI were normal. No other neurological problems emerged during four years of follow-up. This patient experienced two episodes of amnesia, fulfilling the criteria for TGA [3]. On one occasion, the TGA attack ended in an episode of TTA. These appeared as two distinct features, one leading to the other, but each maintaining its own characteristics. During TGA, the patient had a preserved spatial orientation. The topographical disorientation began once the TGA attack subsided and developed the typical course of TTA, with a maintained ability to record the events during the attack. Similarities between TGA and TTA have been previously noted [5, 6]. Both consist of transient memory dysfunction occurring in healthy middle aged or elderly people. Both have a good outcome, leaving at most a mild frailty on circumscribed cognitive performances, such as verbal memory in TGA [1] and geographical orientation in TTA [7]. This observation reinforces the idea of a possible common pathogenesis for TGA and TTA. Current pathogenetic hypotheses for TGA favor functional changes in cerebral metabolism more than structural damage. Leao’s spreading depression, suggested by Olesen and Jorgensen in 1986 [4] to explain TGA, has been recently reconsidered [2]. It is possible that stress, emotional and/or physical experiences may induce biochemical changes in the hippocampus, triggering a spreading depression, leading to a decreased local metabolism as well as a reversible functional ablation of the structures critical for memory. This mechanism could be invoked both for TGA and TTA. Both disorders may be due to the effect of spreading depression, which, propagating in different parts of the brain, may induce varied types of amnesia based on the affected region. LETTER TO THE EDITORS

Late Onset Bipolar Disorder due to a Lacunar State

Objective. To describe a patient with a new onset bipolar disorder (BD) type II, secondary to a lacunar state. Background. Poststroke BD is rare and mainly associated with lesion in the prefrontal-striatal-thalamic circuit. Materials and Methods. A 51-year-old woman came to our attention for a mood disorder of recent onset. At 49, she had suffered acute left-sided limb weakness that improved spontaneously four days later. Arterial hypertension was subsequently diagnosed. After 6 months, she began to suffer from alternating brief periods of expansive and elevated mood with longer periods of depressed mood, with a suicide attempt. We performed extensive laboratory and instrumental investigations, as well as, psychiatric consultation, and a cognitive assessment, which was repeated 9 months later. Results. Brain magnetic resonance disclosed leukoaraiosis and a lacunar state of the basal ganglia. Transcranial Doppler showed a patent foramen ovale. A psychiatric consultation led to the diagnosis of BP type II. Neuropsychological evaluation detected deficits in attention/executive functions, verbal fluency, and memory. Nine months later, after specific psychiatric therapy, the psychiatric symptoms were remarkably improved. Conclusion. Our case sheds light on the role of the basal ganglia in mood disorders and the importance of ruling out brain injury in late onset BP.

Postpneumococcal Moyamoya syndrome case report and review of the postinfective cases

Our aim was to describe a patient who experienced a postpneumococcal Moyamoya syndrome (MMS), with a great involvement of the posterior cerebral circulation, and to review the MMS postinfective cases. A 55-year-old Pakistani man with a history of pneumococcal meningitis 3 months before developed acute headache, left otalgia and body paresthesiae. Brain CT showed a right occipital ischaemic lesion. Seven days later, he developed acute left haemianopsia, haemiplegia, haemineglect and ‘frontal’ cognitive and behavioural symptoms. A second brain CT and MRI disclosed an increase in the occipital lesion and the appearance of a further one in the right frontal lobe. Cerebral CT and MRI-angiography were consistent with Moyamoya vessel alterations. Treatment with antiplatelets, methylprednisolone, followed by prednisone tapering, and motor rehabilitation began. Six months later, no relapses had occurred. Our case represents a delayed manifestation of postmeningitis vasculopathy. Meningitis may represent a risk factor for developing a disabling cerebrovascular disease like MMS.