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Biomedical subjects

P G Rossi

Publications and source records attributed to P G Rossi.

At least 19 recordsLinked to original sources
PubMed2001

Ictal and nonictal paroxysmal events in infantile neuroaxonal dystrophy: polygraphic study of a case.

A 7.5-year-old girl, with infantile neuroaxonal dystrophy (INAD), showed a gradual deterioration from 16 months; at age 5 years she was bedridden, with severe tetraplegia, strabismus, nystagmus and optic atrophy, and dementia. From age 5.5 years, she had paroxysmal tonic events. Videopolygraphic recordings disclosed two different kinds of motor events: (a) epileptic tonic seizures, in wakefulness and sleep, associated with autonomic changes and ictal EEG discharges; and (b) nonepileptic prolonged clusters of brief tonic spasms, without ictal modifications of the EEG. Both motor events were characterized by a minimal and clinically similar tonic contraction of the upper extremities. Video-polygraphic studies are mandatory for a correct paroxysmal event classification and treatment in INAD patients.

Arm↗
PubMed2000

Sneddon syndrome, arylsulfatase A pseudodeficiency and impairment of cerebral white matter.

We describe a 11 year-old-boy with Sneddon syndrome, confirmed by skin biopsy, and MR evidence of diffuse cerebral hyperintensity of white matter; he also suffered from pre-perinatal hypoxic-ischemic distress. Arylsulfatase A activity was found reduced because of arylsulfatase A pseudodeficiency. We suggest that the association of pre-perinatal distress, Sneddon syndrome and arylsulfatase A pseudodeficiency is responsible for the diffuse impairment of cerebral white matter, never reported in Sneddon syndrome and similar to described cases of delayed posthypoxic demyelination and arylsulfatase A pseudodeficiency.

Cerebral Cortex↗
PubMed1999

Landau-Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES).

We describe 11 patients affected by Landau-Kleffner syndrome (LKS) with a mean follow-up of 9 years and 8 months. EEG recordings during wakefulness, NREM and REM sleep showed a bitemporal electrical status epilepticus during sleep (BTESES) in all cases; four of them presented a shift from a BTESES towards an 'intercalated electrical status epilepticus during sleep' (IESES) accompanied by a global regression of cognitive and behavioural functions in 3/4 of cases. At the last observation, only 18.2% of cases presented a complete language recovery and mental retardation was evident in 63.6%. The prognosis of LKS in our cases may depend on the interaction of different negative factors such as onset of aphasia before 4 years, its duration for longer than 1 year, long-lasting duration and continuity without fluctuations of BTESES/IESES, probably preexisting mild speech delay. It is important for the prognosis to utilize antiepileptic treatment and possibly neurosurgical techniques to eliminate EEG paroxysmal abnormalities. At present, no similar cases with clinical-EEG evolution from LKS to electrical status epilepticus during sleep (ESES) have ever been described. Our observation demonstrates that LKS and ESES classified as different clinical-EEG syndromes represent two aspects of the same brain dysfunction and they may exist separately or pass one into the other with a change in the clinical-EEG picture. The common origin of the two syndromes is confirmed by recent functional brain imaging, neurophysiological and neurosurgical techniques.

Adolescent↗
PubMed1999

Bilateral centrotemporal spikes triggered by blinking: an unusual form of sensory input with related cortical EEG activity.

OBJECTIVE: To investigate the morphology, scalp topography and temporal relationship with orbicularis oculi muscle contraction of bilateral blink related spikes (BRS) in a 7-year-old boy with chromosomopathy, mild mental retardation and left spontaneous centrotemporal spikes (SS). METHODS: The patient underwent video-polygraphic recordings with off-line analysis of SS and BRS by means of spike-averaging and orbicularis oculi contraction-locked averaging techniques respectively. EEG activity related to reflex blinking (evoked by glabellar tapping) was also studied. RESULTS: SS and BRS presented the same morphology, characterised by four peaks (P1, N1, P2, N2). SS were located over the left centroparietal regions, while BRS were placed over both left and right centrotemporoparietal regions and constantly followed the contraction of orbicularis oculi with overlapping peak latencies over C3 and C4 electrodes (P1 72 ms; N1 115 ms; P2 164 ms; N2 236 ms). Reflex blinking evoked a small waveform with the same features as BRS. CONCLUSIONS: Our findings suggest that both involuntary and reflex blinking can act as a form of sensory stimulation probably engaging similar nervous pathways and cortical sources in generating EEG abnormalities: the trigeminal system.

Action Potentials↗
PubMed1999

Posterior fossa malformations and epilepsy.

The association between posterior fossa malformations and epilepsy is rarely reported in the literature. We describe 54 cases with posterior fossa malformations, according to embryogenesis classification, divided into two groups on the basis of presence or absence of epilepsy. Epilepsy occurred in 22 cases (40.7%) and was not related to the type of posterior fossa malformation or to supratentorial cerebral lesions associated with the malformation. Familial antecedents for epilepsy and/or febrile convulsions influenced the presence of epilepsy in patients with posterior fossa malformations (P < .01). Epilepsy was mainly partial (77.3%); benign partial/generalized epilepsies and febrile convulsions occurred in 27.3% of cases. Seizures disappeared for 2 or more years at the end of follow-up in 36.4% of patients. Good epilepsy prognosis was not related to the age at onset of seizures, familial antecedents for epilepsy and/or febrile convulsions, supratentorial associated lesions, or age of patients at the last observation. Profound mental retardation prevailed in patients with epilepsy (P < .01), as did pathologic electroencephalograms (EEG) (P < .0001), with paroxysmal abnormalities (P < .001) and asymmetry (P < .01). In our 54 cases of posterior fossa malformation, we identified two risk factors for epilepsy: familial antecedents for epilepsy and/or febrile convulsions and the involvement of the cerebellum in the malformation.

Adolescent↗
PubMed1999

Niaprazine in the treatment of autistic disorder.

Niaprazine is a histamine H1-receptor antagonist with marked sedative properties. It has been employed in subjects with behavior and sleep disorders. No data concerning the use of niaprazine in subjects with autistic disorder are reported in the literature. The authors performed an open study to assess niaprazine efficacy in a sample of 25 subjects with autistic disorder and associated behavior and sleep disorders. Niaprazine was administered at 1 mg/kg/day for 60 days. A positive effect was found in 52% of patients, particularly on hyperkinesia, unstable attention, resistance to change and frustration, mild anxiety signs, heteroaggressiveness, and sleep disorders. Statistical comparison between responders and nonresponders showed no influence on niaprazine effect by age over or under 12 years, presence of neurologic signs, epilepsy, or abnormalities seen on brain imaging. Niaprazine was more efficacious in subjects with a mild or moderate degree of mental retardation. No side effects were observed. Because of its sedative effects and good tolerability, niaprazine can be used as a first-choice drug to improve behavior and sleep disorders in patients with autistic disorder.

Adolescent↗
PubMed1998

Exacerbation of epileptic seizures by carbamazepine: report of 10 cases.

Carbamazepine (CBZ) is an effective anticonvulsant agent. Current literature reports describe several cases of seizure exacerbation and/or EEG worsening due to CBZ with a high prevalence in children and adolescents; we report 10 new cases. Nine patients had epilepsy; one showed delayed psychomotor development and frequent EEG paroxysmal abnormalities. Four patients were on monotherapy, six on polytherapy. All but one had therapeutic CBZ plasma concentrations. Seizures increased in frequency in nine, and in eight patients new seizure types appeared, mostly absences. Cognitive functions/behaviour worsened in eight; EEG recordings showed slowing background activity and increased paroxysmal abnormalities, in six cases diffuse/generalized spike waves were seen and in two continuous spike wave discharges. The mean time of clinical EEG worsening was 1-2 days after introduction of CBZ at therapeutic doses. After CBZ withdrawal clinical EEG improvement was evident in a few days. The underlying pathogenetic mechanism is not yet understood. However, the pathophysiology of seizure exacerbation might be related to the interaction between age-related alterations in the balance of excitation and inhibition in the developing thalamocortical circuitry and the essential activity of CBZ that tends to induce interictal discharges.

Age Factors↗
PubMed1997

Benign myoclonic epilepsy: long-term follow-up of 11 new cases.

The authors report a long-term follow-up of 11 new subjects with benign myoclonic epilepsy. There were some unusual clinical features such as the need for dual therapy in 45.5% of subjects, and the presence of non-epileptic myoclonus in 54.5%, neither of which influenced the prognosis. Neuropsychological and behavioral evolution was less favorable in 45.5% of patients (mental retardation, school learning problems, attention deficit disorder, hyperkinesia, aggressiveness, irritability, negativism). The less favorable neuropsychological outcome might be related to additional interacting factors such as personal antecedents, seizure onset and antiepileptic treatment.

Adolescent↗
PubMed1997

Familial Unverricht-Lundborg disease: a clinical, neurophysiologic, and genetic study.

PURPOSE: Progressive myoclonus epilepsies (PMEs) are a clinically and etiologically heterogeneous group of disorders. The authors report clinical, neurophysiological, and genetic findings of a family from Southern Italy with three members affected with PME. METHODS: All data about familial and personal antecedents, clinical history, neurologic examination, laboratory tests, neurophysiological findings, brain imaging studies, and DNA analysis were examined. RESULTS: All results were compatible with the features of Unverricht-Lundborg disease and patients were homozygous for the "Finnish" ancestral haplotype. CONCLUSIONS: Work is in progress to identify and characterize the common EPM1 mutation in the Finnish patients. Subsequently, it will be possible to verify the hypothesis on the existence of a common mutation in the Finnish patients and the Italian family under study, or even in other Mediterranean EPM1 families.

Adult↗
PubMed1995

Possible interaction between acyclovir and antiepileptic treatment.

We report a clinically relevant suspected interaction between acyclovir and the antiepileptic drugs phenytoin (PHT) and valproic acid (VPA). In a child receiving PHT and VPA therapy, a 6-day acyclovir treatment reduced PHT and VPA plasma concentrations to subtherapeutic values. This probably worsened both clinical status and electroencephalographic recordings observed in this patient. We suggest that the interaction occurs at gastrointestinal level with a reduction of PHT and VPA oral bioavailability during antiviral treatment.

Acyclovir↗
PubMed1992

Neurological impairment in congenital bilateral ptosis with ophthalmoplegia.

A case is described of congenital bilateral ptosis and ophthalmoplegia due to incomplete bilateral paralysis of the third cranial nerve associated with dysmorphisms, brain malformations and epileptiform EEG abnormalities. We hypothesize that in our case the ophthalmological disturbance is due to mesencephalic impairment. In literature there are few reports of congenital bilateral paralysis of the third cranial nerve and they lack detailed MRI findings. We stress in patients with congenital third cranial nerve palsy the importance of thorough neurological investigations including prolonged wake-sleep EEG monitoring as well as CT scan and MRI to establish the origin of the disorder.

Blepharoptosis↗
PubMed1988

Epilepsy with bilateral occipital calcifications: a benign onset with progressive severity.

We studied four patients with a focal epilepsy and bilateral occipital corticosubcortical calcifications without any sign of phakomatosis. The clinical course of the disease was similar in all the patients and evolved from a benign onset to a severe encephalopathy with progressive mental impairment. The question of whether these patients have an incomplete and atypical form of Sturge-Weber syndrome or a previously undescribed disorder is addressed.

Adolescent↗
PubMed1986

L-5-hydroxytryptophan versus placebo in childhood migraine prophylaxis: a double-blind crossover study.

L-5HTP was tested versus placebo in a double-blind crossover study of 27 migraine children aged 6-12 years, who recorded their headaches in a headache diary for 1 month. Twenty-one patients subsequently started the trial. The mean daily dose of L-5HTP was 5 mg/kg body weight, and each treatment period with either L-5HTP or placebo lasted 12 weeks. In group A (L-5HTP-placebo; 10 patients) and group B (placebo-L-5HTP; 11 patients) both L-5HTP and placebo led to a significant reduction of the migraine index and frequency of migraine attacks during the 3rd month of each treatment period. However, we found a treatment X period interaction because the efficacy determinants decreased significantly during the first and the second treatment periods in both groups irrespective of the sequence of treatments. No differences were found between L-5HTP (first period of group A) and placebo (first period of group B).

Child↗
PubMed1985

Occipital seizures in Lafora disease: a further case documented by EEG.

The authors present the case of a patient with Lafora disease proven by skin biopsy, who suffered two occipital seizures recorded on EEG and provoked by intermittent photic stimulation. On the basis of data in the literature and their present experience, the authors suggest the involvement of the occipital lobes as a whole in the epileptogenic activity.

Adolescent↗
PubMed1984

Infantile behavioural regression and respiratory impairment.

Three girls showed psychomotor regression arising between the 1st and 2nd year of life, after an initially normal development. Language, gait, chewing and swallowing were especially affected, whereas elementary sensory and motor functions were relatively spared. All displayed a particular disorder of breathing, which was irregular and inadequate during wakefulness, returning to normal during sleep. There are similarities between these patients and the syndrome of cerebral atrophy with hyperammonaemia, even though brain CT and biochemical studies, including ammonaemia, were all normal.

Adolescent↗
PubMedDate not supplied

Interictal EEG findings in two cases with 'double cortex' syndrome.

'Double cortex' is a neuroblast migrational disorder characterized by a diffuse band of heterotopic grey matter between the lateral ventricles and cerebral cortex which may be normal or macrogyric. The authors report two girls with 'double cortex' syndrome presenting intractable partial epilepsy and severe mental retardation. EEG data are analysed in detail because such patients presented a particular interictal EEG background activity, not only with very stable features during the different stages of vigilance, but also uninfluenced by seizure frequency or duration. The authors raise the possibility that a further in vivo diagnostic parameter for this syndrome has been identified.

Adolescent↗
PubMedDate not supplied

EEG features and epilepsy in patients with autism.

Epileptic seizures are frequently reported (4-32%) in autism. These values are higher than in the normal population of children and adolescents (0.5%). In the literature there is no uniform description of epilepsy in autism. We examined 106 patients with autistic disorder divided into three groups on the basis of presence or absence of EEG paroxysmal abnormalities (PA) and / or epilepsy including febrile convulsions (FG). Our patients presented an autistic syndrome unrelated to clear congenital or acquired encephalopathy. The prevalence of epilepsy and EEG PA was 23.6% and 18.9%, respectively. Significant differences between the three groups appeared for (i) familial antecedents for epilepsy / FC and neurologic and psychiatric diseases (P < 0.004), (ii) a different proportion between the three groups for mental retardation (P < 0.03), (iii) and EEG fast activity (P < 0.04). Our patients showed several types of epilepsy, including idiopathic forms with seizure onset after the age of 10 in 45% of cases. Seizures were mainly partial, not frequent and controllable by anti-epileptic drugs. PA were mostly focal and multifocal and in 45% of cases were typical of benign childhood partial epilepsy with centro-temporal spikes. The higher incidence of epilepsy and EEG PA is apparently not related to organic pre-, peri- and postnatal antecedents or cerebral lesions. On the contrary, genetic factors responsible for autism and epilepsy seem important in the genesis of these two disorders.

Adolescent↗