Maria Guarino

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.

The THRombolysis and STatins (THRaST) study

Objective: To assess the impact on stroke outcome of statin use in the acute phase after IV thrombolysis. Methods: Multicenter study on prospectively collected data of 2,072 stroke patients treated with IV thrombolysis. Outcome measures of efficacy were neurologic improvement (NIH Stroke Scale [NIHSS] ≤ 4 points from baseline or NIHSS = 0) and major neurologic improvement (NIHSS ≤ 8 points from baseline or NIHSS = 0) at 7 days and favorable (modified Rankin Scale [mRS] ≤ 2) and excellent functional outcome (mRS ≤ 1) at 3 months. Outcome measures of safety were 7-day neurologic deterioration (NIHSS ≥ 4 points from baseline or death), symptomatic intracerebral hemorrhage type 2 with NIHSS ≥ 4 points from baseline or death within 36 hours, and 3-month death. Results: Adjusted multivariate analysis showed that statin use in the acute phase was associated with neurologic improvement (odds ratio [OR] 1.68, 95% confidence interval [CI] 1.26–2.25; p < 0.001), major neurologic improvement (OR 1.43, 95% CI 1.11–1.85; p = 0.006), favorable functional outcome (OR 1.63, 95% CI 1.18–2.26; p = 0.003), and a reduced risk of neurologic deterioration (OR: 0.31, 95% CI 0.19–0.53; p < 0.001) and death (OR 0.48, 95% CI 0.28–0.82; p = 0.007). Conclusion: Statin use in the acute phase of stroke after IV thrombolysis may positively influence short- and long-term outcome.

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.

An epidemiological study on severe mental retardation among schoolchildren in Bologna, Italy.

The prevalence, causal origin of and impairments associated with severe mental retardation (SMR) were investigated among all school‐age children (six to 13 years) living in the city of Bologna, Italy. 90 children (57 boys, 33 girls) with IQs ≤ 50 were identified. The prevalence of SMR was 4‐2 per 1000 for males, 2–5 per 1000 for females and 3–4 per 1000 for both sexes. Causal origin was prenatal for 33‐3 per cent, perinatal for 14‐4 per cent, combined pre‐ and perinatal for 5–6 per cent and postnatal for 13‐3 per cent. Another 12‐3 per cent of the children with IQs ≤ 50 had autism or childhood psychosis, while there was no evident cause of mental retardation for the remaining 21‐1 per cent. 50 per cent had at least one associated physical or neurological impairment other than mental retardation, with epilepsy and cerebral palsy predominating.

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.

Risk of seizures while awake in pure sleep epilepsies A prospective study

Objective: To estimate the risk of seizures while awake in pure sleep epilepsies in a prospective study. Methods: From October 1, 1992, to October 31, 1996, all patients with pure sleep epilepsy presenting at a participating center were enrolled. Children with benign rolandic epilepsy and patients with frontal lobe epilepsy were excluded. Patients were followed for at least 2 up to 6 years. The primary endpoint was the occurrence of a seizure while awake. Results: The authors enrolled 161 patients (64% male). Age at the time of inclusion ranged from 11 to 83 years (mean 43.2, median 39). Eighty-five percent presented generalized tonic clonic seizures. Both sleep seizures and seizures while awake were absent for 2 years after inclusion in the study in 78% of patients. Eighteen patients presented a seizure while awake. The estimated risk of a seizure while awake during 6 years of follow-up was 13% (95% CI 7 to 18%). Multivariate analysis showed that episodes of sudden withdrawal of therapy and a higher frequency of seizures at inclusion were associated with an increased risk of seizures while awake. The estimated risk of a seizure while awake in patients with none of the above risk factors was 6.5% (95% CI 1.5 to 11.3%) during 6 years of follow-up. Conclusion: The clinical picture of pure sleep epilepsies is characterized by a preponderance of generalized tonic clonic seizures, low seizure frequency, and a good prognosis. The risk of occurrence of a seizure while awake is low, particularly among patients with rare seizures and good compliance with the therapy.

PREVALENCE OF MENTAL RETARDATION RELATED TO FRAGILE X SYNDROME AND OTHER CHROMOSOMAL ABNORMALITIES THE REPUBLIC OF SAN MARINO

The prevalence of mental retardation related to chromosomal abnormalities, including fragile × syndrome, was studied among 2735 males aged between five and 20 years living in the Republic of San Marino. Five cases of chromosomal abnormalities were found, including one case with fragile × syndrome, with partial epilepsy and a CT scan showing a large arachnoid cyst in the posterior fossa. The prevalence of mental retardation due to chromosomal abnormality was 1 · 83 per 1000, and that due to fragile × syndrome was 0 · 37 per 1000. Although these findings relate to only a small population, they are the first from a Mediterranean area.

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.