Fatal cerebral haemorrhage after liver transplantation in a patient with transthyretin variant (gly53glu) amyloidosis.

Transthyretin (TTR) amyloidosis is a form of systemic amyloidosis caused by an amyloidogenetic TTR variant. The most common mutant forms of TTR are mainly produced by the liver and therefore orthotopic liver transplantation (OLT) is currently accepted as the only known curative treatment. We describe the clinical and pathological features of a patient with TTR variant (gly53glu) with TTR amyloid infiltration of the leptomeningeal vessels in whom fatal cerebral haemorrhage occurred two months after OLT, soon after severe viral pneumonia.

Epidemiology of status epilepticus in a rural area of northern Italy: a 2-year population-based study.

We performed a 2-year population-based study on status epilepticus (SE) in adults in the rural area of Lugo di Romagna, northern Italy, to verify whether an area of low-level urbanization has a lower risk of occurrence of SE (as recently suggested), different clinical features and short-term prognosis than areas of high-level urbanization. We found crude and age- and sex-adjusted annual incidence rates of SE of 16.5/100 000 and 11.6/100 000, respectively. In patients under 60 years crude incidence was 2.9/100 000 and in the elderly (>/=60 years) 38.6/100 000. Acute symptomatic SE accounted for 30% and a cerebrovascular pathology was the most frequently associated etiologic condition (60%). A history of seizures was reported in 41% of patients. The first therapeutic intervention was mainly benzodiazepines (lorazepam 46%; diazepam 33%). The 30-day case fatality was 7%. We observed that the adult population of an area with a low level of urbanization has the same risk for SE, clinical features and short-term prognosis as European urban areas. The only contrasting result is the 30-day case fatality of 7% against the 39% found in the other Italian study (Bologna), despite the similarity of the SE features in these two areas of the same region. We infer that the short-term prognosis of SE could also be considerably influenced by differences in health service organization (and hence management) possibly due to different levels of urbanization.

Accuracy of ICD-9 codes in identifying ischemic stroke in the General Hospital of Lugo di Romagna (Italy).

We assessed the sensitivity and the positive predictive value (PPV) of the ICD-9 codes in identifying ischemic strokes. The study involved the cross-sectional comparison between patients with an ischemic stroke diagnosis made by neurologists and patients with the 434 or 436 discharge codes. Sensitivity of the codes (all diagnostic levels and first level respectively) was 82% and 76%; PPV: 71% and 76%. The annual crude incidence of ischemic stroke was 2.62 per 1000 based on verified strokes and 3.03 per 1000 based on 434 or 436 coded medical records (at all diagnostic levels). Thirty-day case fatality ratio was 22.3% in verified strokes and 36.8% among patients diagnosed with codes 434 or 436 but without stroke (all levels). Our results disclosed inaccuracy in use of the ICD-9 codes in the diagnosis of ischemic stroke in the general hospital of Lugo di Romagna, Ravenna Province, Italy. The misdiagnosis of patients could be influenced by the degree of severity of clinical features. Epidemiological data and cost-analysis forecasts based only on the ICD-9 system must be considered with caution.

Status epilepticus in acute ischemic stroke.

Stroke is one of the most frequent causes of acute symptomatic status epilepticus. The aim of this study was to investigate the electroclinical features of status epilepticus in acute ischemic stroke. Nine consecutively admitted patients with status epilepticus during ischemic stroke were examined: five of them had convulsive unilateral or generalized status epilepticus for from 24 hours to 9 days after a large hemispheric infarction, always associated with EEG epileptiform abnormalities; the remaining four had focal motor status epilepticus during the first 24 hours after a small cortical or subcortical infarction, and showed no clear EEG changes. Status epilepticus in acute ischemic stroke may have two distinct electroclinical patterns of different prognostic significance.

Acute psychosis and epileptic seizures as the presenting symptom of late-onset epilepsy.

We report two patients with no history of psychosis or epilepsy presenting with acute psychosis and epileptic seizures as the initial symptom of late-onset epilepsy. Seizures and psychosis resolved with phenytoin. Case 1 presented a recurrence of both psychosis and seizures following abrupt withdrawal of antiepileptic drugs.

Brain energy metabolism studied by 31P-MR spectroscopy in a case of migraine with prolonged aura.

The brain and skeletal muscle oxidative metabolism of a patient with prolonged aura was studied by phosphorus magnetic resonance spectroscopy. We found that the phosphocreatine to ATP ratio in brain was reduced, while the inorganic phosphate to phosphocreatine ratio and the calculated ADP concentration were increased. The phosphorylation potential and percentage of maximal rate of ATP synthesis were also altered. Intracellular pH and inorganic phosphate concentration were normal. In muscle we found a low post-exercise recovery of phosphocreatine. These data indicate an impairment of energy oxidative metabolism both in brain and muscle.

Transient abnormalities on magnetic resonance imaging after partial status epilepticus.

We report two patients who developed focal abnormalities on MRI after partial status epilepticus. Maximum radiological modification occurred in the area of maximal epileptic discharge. Subsequent MRI failed to demonstrate persistent abnormalities. These transient abnormalities on MRI could be an expression of cerebral edema caused by focal epileptic status.

Migraine with aura and photosensitive epileptic seizures: a case report.

An 18-year-old female presented with two seizures induced by photic stimulation. She had a positive family history for migraine and a history of febrile convulsions. Since the age of 13 she had suffered from migraine attacks with aura. A brain computerized tomography with contrast enhancement was negative and several electroencephalograms showed a photoparoxysmal response. At the age of 18 she had a partial secondary generalized seizure after photic stimulation during routine electroencephalogram. The onset of seizure was in the occipital region. Two days later, the patient presented with a typical migrainous attack with aura. Interictal apomorphine test (1.5 mg s.c.) blocked the photoparoxysmal response. According to Quesnay, dopaminergic failure of the occipital cortex may account for both epileptic and migraine features.

Electroencephalogram and HIV infection: a prospective study in 100 patients.

In order to study the correlation between central nervous system (CNS) involvement and EEG abnormalities in HIV infection we studied 100 consecutive HIV patients. Patients were divided into 4 groups; Group I: 42 neurologically asymptomatic subjects; Group II: 6 patients with peripheral neuropathies; Group III: 28 patients with AIDS Dementia Complex; Group IV: 24 patients with secondary CNS involvement. The results of this study emphasize that abnormal EEGs are correlated with CNS involvement. Neurologically asymptomatic patients showed no abnormal tracings, but the presence of borderline EEGs (33%) in asymptomatic patients should be evaluated prospectively.

Ischemic stroke in young adults: the relevance of migrainous infarction.

Sixty-one consecutive patients, less than or equal to 40 years old, were hospitalized for cerebral infarction between 1977 and 1985. Evaluation included computed tomographic brain scan, arteriography, echocardiography, and blood tests. A probable migrainous infarction was diagnosed in six patients (10%) (all women with a history of migraine) who survived the initial stroke and were followed-up for an average of four years. In five patients the stroke occurred during a common migraine attack and in one patient during a classic migraine attack. The site of infarction was invariably the occipital lobe. During the follow-up, no subject had a further stroke. All six women had a permanent hemianopic deficit.

Normal muscle mitochondrial function in Ramsay-Hunt syndrome.

Mitochondrial encephalomyopathies may display clinical features similar to Ramsay-Hunt syndrome (RHS). We studied muscles mitochondrial function in 2 patients with RHS. Histochemical and ultrastructural studies of muscle biopsies and biochemical analysis of muscle mitochondrial enzymes were normal. There is no evidence for a disturbance of muscle mitochondrial function in RHS.

Epidemiology of headache in the Republic of San Marino.

An epidemiological survey on headache was performed in the Republic of San Marino, which is the smallest independent State in the world, located near the Adriatic Coast, within Italy. Among a random sample of 1500 inhabitants over 7 years of age the frequency of headache, severe headache and migraine in the previous year was 35.3%, 12.2%, 9.3% respectively for men, and 46.2%, 20.6%, 18% for women. The most common factors reported to provoke headache were emotional stress, physical strain, lack of sleep, particular foods or drinks and for women menstruation. Migraine patients differed from people without headache in that they had a higher consumption of coffee, more frequently reported bad sleep, allergic disease and previous appendectomy. Furthermore, migraine patients and severe headache sufferers had a higher diastolic blood pressure than non headache subjects.