Levetiracetam versus carbamazepine in patients with late poststroke seizures: a multicenter prospective randomized open-label study (EpIC Project).

BACKGROUND: Strokes are the leading cause of epileptic seizures in adults and account for 50% of seizures in those over the age of 65 years. The use of antiepileptic drugs to prevent recurrent poststroke seizures is recommended. METHODS: One hundred and twenty-eight patients with poststroke seizures were randomly allocated to treatment with either levetiracetam (LEV) or sustained-release carbamazepine (CBZ) in a multicenter randomized open-label study. After a titration study phase (2 weeks), the optimal individual dose of trial medication was determined and treatment was continued for another 52 weeks. The primary endpoint was defined as the proportion of seizure-free patients; the secondary endpoints were: evaluation of time recurrence to the first seizure, EEG tracings, cognitive functions and side effects. RESULTS: Of 128 patients, 22 discontinued the trial prematurely; thus a total of 106 patients (52 treated with LEV and 54 treated with CBZ) were included in the analysis. The results of the study were as follows: no significant difference in number of seizure-free patients between LEV and CBZ (p = 0.08); time to the first recurrence tended to be longer among patients on LEV; there was no correlation between the therapeutic effect and the EEG findings in either treatment group; LEV caused significantly fewer (p = 0.02) side effects than CBZ; attention deficit, frontal executive functions and functional scales (Activities of Daily Living and Instrumental Activities of Daily Living indices) were significantly worse in the CBZ group. CONCLUSIONS: This trial suggests that LEV may be a valid alternative to CBZ in poststroke seizures, particularly in terms of efficacy and safety. In addition, our results show that LEV has significant advantages over CBZ on cognitive functions. This trial also indicates that LEV in monotherapy is a safe and effective therapeutic option in elderly patients who have suffered epileptic seizures following a stroke.

Juvenile myoclonic epilepsy with generalised and focal electroencephalographic abnormalities: a case report with a molecular genetic study.

This is the case of a 16-year-old girl with juvenile myoclonic epilepsy (JME) and maternal family history positive for epilepsy and febrile seizures, presenting ictal and interictal generalised, as well as focal paroxysmal abnormalities over the right central-temporal regions activated during sleep. The brain magnetic resonance image was normal and the seizures responded to therapy with valproate and lamotrigine. A molecular genetic analysis led to the identification of a polymorphism (A-->G) in position 10 in the intron 3 (rs949626) of the EFHC1 gene; and a polymorphism (T-->C) of the exon of the GABRA1 gene, without aminoacidic exchange. In the literature this is the first case of JME with electroencephalograph focal epileptiform abnormalities, but without EFHC1 and GABRA1 gene mutations.

Association in the same patient of autosomal dominant progressive external ophthalmoplegia with multiple mtDNA deletions and X-linked ichthyosis: clinical, biochemical, histological, submicroscopic and molecular genetic study.

Autosomal dominant chronic progressive external ophthalmoplegia (AdPEO) is a muscle mitochondrial disorder due to multiple large scale rearrangements of the mitochondrial DNA. This disorder is probably due to a nuclear defect which causes genetic instability or an impairment in the replication of mitochondrial DNA. X-linked ichthyosis (XLI) is a skin disorder caused by a deletion in the steroid-sulphatase gene. Here we report the clinical, biochemical, morphologic and molecular genetic findings in a patient affected by both AdPEO, inherited by the father, and steroid-sulphatase-deficiency, inherited by the mother. The association in the same patient of the two inherited diseases is merely casual and does not seem to influence the phenotypic expression of the two diseases.

Electrophysiological and neuropsychological functions in patients treated with interferon-beta.

The neurotoxicity of interferon-beta (IFN-beta) was assessed by performing electrophysiological examinations and neuropsychological tests on 22 patients with malignant hematological diseases before, during, and after IFN-beta treatment. IFN-beta (6 x 10(6) IU/m2) was infused i.v. for 6 h daily for 7 days on alternate weeks for a total of three cycles (induction therapy) and was then continued at the same dose, twice a week, for an additional 24 weeks (maintenance therapy). Twenty-one of the 22 patients were evaluable. There were no significant changes in EEGs, visual evoked potentials, sensory conduction central time or motor nerve conduction velocity of two long nerves in the 15-19 patients studied before and after induction therapy, nor in the 6-8 patients investigated at the end of maintenance therapy. Neuropsychological monitoring failed to disclose any IFN-induced deterioration in 21 patients tested before and at the end of induction therapy or in the 10 patients who were also studied at the end of maintenance therapy. Despite certain limitations in the patient follow-up, the results underline the good general tolerance of IFN-beta.

Acute neurotoxicity after trichloroethylene ingestion. Case report.

Trichloroethylene (TCE), a solvent widely used in the chemical industry, in dry cleaning because of its degreasing action and as a household grease remover, is known to have a toxic action, especially on the nervous system. Cases of intoxication, acute and chronic, due to inhalation, are reported. We report a case, certainly an unusual one, of acute oral intoxication.

Neuralgic amyotrophy of the shoulder girdle. The Parsonage-Turner syndrome.

From 1971 to 1983 we observed 58 cases of the Parsonage-Turner syndrome. This is a neuromuscular disease of the shoulder girdle and upper extremity which up to now has been dealt with almost exclusively in the French and Anglo-Saxon literature. The aetiology remains obscure. It is characterised by pain of acute and violent onset with paralysis and wasting of the muscles of no specific nerve root distribution. The diagnosis is based on clinical examination and electromyography. The prognosis is usually favourable. The differential diagnosis and treatment are discussed.

Progressive bulbar paralysis in childhood: a case report.

The case of a progressive bulbar paresis in a nine and a half year old child is reported. The first symptoms were present at birth; however, the subsequent evolution was very low. Lesion of the motor nuclei of the V, VII, IX, XII, cranial nerves was evident on electromyographic investigation. Damage to the acoustic brain stem pathway was documented by the brain stem evoked potentials although audiometry was normal. No other neuronal systems or districts appeared to be damaged. The case suggests Fazio-Londe disease, although the involvement (albeit partial) of the auditory pathways recalls Van Laere syndrome. This supports the view that motor neuron disease in infancy is not an autonomous entity but a variant in a wide spectrum of progressive neuronal diseases.

[The problem of Parkinsonism associated with the motor neuron disease. Clinical report on 11 sporadic cases (author's transl)]. / Il problema della associazione fra sindrome parkinsoniana e sclerosi laterale amiotrofica. Rilievi clinici in 11 casi sporadici.

The clinical data are presented of 11 subjects affected by a sporadic form of Parkinsonism with associated motor neuron disease. Combined lesions of upper and lower neuron were found in 10 of 11 cases, while in the remaining case only lower motor neuron disorder was present. The female/man ratio is 1/11. The early symptoms appear at a mean age of 56.9 years: in 6 patients with both syndromes at the same time while in 4 only with Parkinson. These observations confirm the view that different degenerative neurological syndromes must not be considered autonomous entities, but only different forms of polysystemic degenerative diseases that choose, in particular conditions, some neuronal districts rather than others.

[Uremic neuropathy. Study of the motor conduction rate in patients treated with maintenance hemodialysis and peritoneal dialysis]. / La neuropatia uremica. Studio della velocità di conduzione motoria in pazienti sottoposti a trattamento emodialitico e dialitico peritoneale di mantenimento

Forty-four patients, treated with different systems of dialysis (peritoneal dialysis, two-weekly and three-weekly hemodialysis) have been controlled by electroneurographic, electromyographic and ematochimical examinations during a six months to five years period. The results can be summarized as follow: a) motor nerve conduction velocity of the peroneal nerve is reduced proportionally to the clinical status in all patients. Motor nerve conduction velocity of the ulnar nerve is reduced only in the most severe cases; b) there are no significant correlations between the examined ematochimical data and the maximal motor nerve conduction velocity; c) there is a slight improvement of the maximal motor nerve conduction velocity of the peroneal nerve when passing from a twelve hours two weekly dialytic treatment to a five hours three weekly hemodialytic treatment. Motor nerve conduction velocity is therefore a reliable index of insufficient or adequate dialysis. Moreover it is confirmed the efficacy of a treatment with short and frequent dialytic sessions.

Toxic polyneuropathy of shoe-industry workers. A study of 122 cases.

The toxic polyneuropathy observed in a group of shoe-industry workers in Italy was clinically characterised by a symmetrical prevalently distal motor deficit, with occasional marked weakness of pelvic girdle muscles, and frequently by only subjective sensory symptoms; non-specific disturbances usually preceded neurological signs. Subclinical cases of 'minimal' chronic neuropathy, characterised by alterations of a neurogenic type in the EMG without a reduction of motor nerve conduction velocity, were also observed. Worsening of the clinical picture, with further lowering of nerve conduction velocity, was noted in some cases up to four months after removal from the toxic environment. In the most severe cases clinical recovery took up to three years. The electromyographic and electroneurographic features were consistent with a mixed axonal neuropathy, with clear prevalence of the damage in the distal part of the nerve (dying-back neuropathy). Volatile substances, such as n-hexane and other low boiling point hydrocarbons found in high percentage in solvents and glues, are suggested as the causative agent.

Shoe-makers' polyneuropathy in Italy: the aetiological problem.

Since 1957, when the first cases of the so-called shoe-makers' toxic polyneuropathy were reported, nearly 400 cases have been described in the Italian literature. The substance that was considered to be responsible for the disease was triorthocresylphosphate (TOCP) contained in glues, artificial leathers, and some types of paints. However numerous chemical analyses of glues and leathers taken from factories where cases of the disease occurred have shown that in almost all instances little or no TOCP was present. In addition the disease manifests itself more frequently during winter and spring and this is not consistent with the absorption of the causative agent through the skin and/or gastrointestinal tract. In order to clarify the aetiology of the disease 122 workers in the shoe industry affected by toxic polymeuropathy during the period 1971-74 were studied. Workplaces where cases of polyneuropathy had occurred were visited and samples of glues and solvents were taken for chemical analysis. The polyneuropathy chiefly affects workers engaged in glueing and in the cleaning process, but it also affects those who do not have direct contact with glues or solvents. The disease is more common in women (68%) than in men (32%) and, in its more severe form begins with overwhelming prevalence during winter and early spring regardless of the job performed. No direct relationship has been found between the severity of the disease and the number of years of work in the shoe industry or of specific exposure (that is the number of years of work in the activity performed at the onset of the disease). The polyneuropathy is found almost exclusively among workers of the artisam type in small shoe factories, where standards of hygiene are low. Chemical analysis of glues and cleaning fluids collected from five different factories, where 20 cases of polyneuropathy occurred, showed the presence of paraffin hydrocarbons with a low boiling point (pentane, 2-methyl-pentane, 3-methyl-pentane, n-hexane, etc.) in concentrations of more than 80% by weight.