Decrease in cerebellar blood flow in patients with Friedreich's ataxia: A TC-HMPAO SPECT study of three cases.

Three cases of Friedreich's ataxia were submitted to diverse neuroradiological procedures in order to determine the extent of atrophic processes in the central nervous system. All patients underwent computerized-tomography scan, Magnetic Resonance Imaging, and HMPA-single Photon emission computerized tomography studies, focusing in cerebellar lobes. A slight atrophy was observed in the vermis and the cerebellar lobes with CT scan and MRI. In contrast a significant decrease in cerebellar blood flow was shown by TC-HMPAE SPECT study. The significance of these findings in understanding physiopathological mechanisms in Friedreich's ataxia is discussed.

Depression and partial epilepsy: relevance of laterality of the epileptic focus.

In a prospective study of 50 epileptic patients (20 with a left hemisphere epileptic focus, 20 with a right one and 10 with a cryptogenic generalized epilepsy), the authors show that depression as appreciated by the H.A.R.D. score, is more frequent and severe in partial epilepsy in males, and when the epileptic focus is localized on left hemisphere. This association is related to the duration of epilepsy, the number of seizures, but not to age or medication status. Vulnerability of left hemisphere to depression is analysed, and neuro-chemical mechanisms are discussed.

[REM-sleep behavior disorder and olivo-ponto-cerebellar atrophy: a case report]. / Trouble du comportement du sommeil paradoxal et atrophie olivo-ponto-cérébelleuse: un cas.

The authors report the observation of REM-sleep behavior disorder in a patient also suffering from an olivo-ponto-cerebellar atrophy. They discuss the place of this sleep disorder among the parasomnias, its pathophysiological basis and its features similar to those of the somnambulism. Only a polysomnography study can help to make the diagnosis.

[Electrophysiologic investigation of the anterior temporal and frontal lobes: sphenoidal and intraorbital electrodes]. / L'exploration électrophysiologique de la face basale du lobe temporal et du lobe frontal: intérêt des électrodes sphénoïdales et intraorbitaires.

The authors have evaluated the interest of sphenoidal electrodes in detection of internal temporal spikes, and intra-orbital electrodes in the detection of orbito-frontal spikes. From a study of 26 patients, 21 with sphenoidal electrodes, 3 with intra-orbital electrodes and 2 with both electrodes, they observed the sensitivity and specificity of such electrodes in detecting spikes with no traduction upon extra-cranial electrodes, or with an unsuspected traduction as spikes at a distance from deep electrodes, or spikes on 2 foci, or bisynchronous discharges. Sphenoidal and intra-oribital electrodes constitute a non-invasive method that provides excellent information in the exploration of the mesiobasal cerebral face. Indications for the use of such a method are complex absences without EEG traduction or with an unsuspected traduction and without abnormalities on CT scan, in the context of functional surgery of epilepsy.

[Value of sphenoidal electrodes in the EEG diagnosis of herpes encephalitis]. / Intérêt des électrodes sphénoïdales dans le diagnostic EEG des encéphalites herpétiques.

EEG features in acute necrotizing encephalitis show periodic activity, localized in the temporal area with a slow background activity. Nevertheless, this periodic activity is transient and may lack. Sphenoidal electrodes study of the internal face of the temporal lobe could record such activity in herpes encephalitis. Sphenoidal electrodes were inserted in 4 patients known to have herpes encephalitis. In 2 cases, periodic discharges were observed both on the surface and sphenoidal electrodes, while in 2 cases, periodic discharge was observed under only sphenoidal electrodes. Faced with uncertain diagnosis of herpes encephalitis, it may be useful to insert sphenoidal electrodes to record deep periodic discharges, not seen on surface electrodes, in order to confirm the diagnosis.

[Depression and temporal epilepsy. The possible role of laterality of the epileptic foci and of gender]. / Dépression et épilepsie temporale. Rôle possible de la latéralité du foyer épileptique et du sexe.

From a prospective study of 47 epileptic patients (26 men and 21 women) 25 with a left and 22 with a right temporal epileptic focus, the authors show that depression measured by the HARD score, is more severe in males, with a left epileptic focus. These results and the vulnerability to depression of left temporal lobe epileptic patients are analyzed.

[Acute anterior bi-opercular syndrome of critical origin in epilepsy with rolandic spikes]. / Syndrome bi-operculaire antérieur aigu d'origine critique dans l'épilepsie à paroxysmes rolandiques.

We report the fifth case of a palsy of the lips, the tongue and the pharynx corresponding to an acute pseudo-bulbar syndrome causing speech arrest, and hyper-sialorrhea. The clinical examination and the electroencephalograms showed a partial motor status with spikes discharges in the two central regions, in a 10-year old boy known to have epilepsy with rolandic spikes. The status epilepticus ceased with phenytoin therapy. Although epilepsy with rolandic spikes is a benign one without any cerebral lesion, a partial motor status epilepticus is possible and does not change prognosis.

[Agenesis of the corpus callosum and epilepsy. 26 cases]. / Agénésie du corps calleux et épilepsie. 26 cas.

Agenesis of the corpus callosum is a cerebral malformation diagnosed by CT scan. Beside mental retardation, the most frequent clinical manifestation is epilepsy. In a personal study of 26 cases, the authors analysed the clinical features of epileptic seizures observed with this malformation. They put the stress on the paradoxical presence of generalized seizures.

[Value of local cerebral hypoperfusion in the diagnosis of frontal syndromes. Importance in medical expert assessment of head injuries]. / L'apport de l'étude des débits sanguins locaux dans le diagnostic des syndromes frontaux. Son intérêt dans les expertises médicales des traumatisés du crâne.

The importance of the symptoms linked to a frontal disorder in the intellectual and behavioral sequelas of severe cranial trauma, is now measured carefully because these troubles are one of the most important obstacle to the social and professional come-back. The handicap linked to a disorder of the frontal lobe is not well known in all its neurological, mental and sociological dimensions because the diagnosis is not easy. To help to this diagnosis, the study of cerebral blood flow with SPECT is usefull in medical practice. We present here 4 clinical cases of post-trauma frontal syndrome not easy to measure. With a measure of cerebral blood flow with SPECT, it was observed a decrease of frontal blood flow, that allowed to link the neuropsychological symptom to a frontal lobe disorder. It seems that in these conditions the post-trauma frontal syndrome is not so uncommon.

[Effects of callosotomy in the treatment of intractable epilepsies in children on psychiatric disorders]. / Les effets de la callosotomie dans le traitement de l'épilepsie rebelle de l'enfant sur les troubles psychiques.

Two children, 13 and 14 years old, presented an intractable epilepsy of Lennox-Gastaut. In front of dangerous tonic and atonic epileptic crisis, a corpus callosotomy was performed. Corpus callosotomy is becoming a more widely used procedure in the treatment of intractable epilepsy as Lennox-Gastaut syndrome, or frontal epilepsy. However, there have been very few series that have reported results in children. It is not a complete but a partial, callosal section including approximately the anterior two thirds of the callosum. After this surgery the two children improved dramatically because the dangerous tonic and atonic crisis disappeared. Secondly we observed improvement of pre-surgical psychiatric troubles, that is a data not developed in the literature. Before surgery, the two children had a frontal syndrome with hyperkinesia, distractibility, aggressiveness, alexithymia, loss of the program of ideas. During the two months after the section of the anterior two thirds of the corpus callosum, we observed a progressive improvement of the frontal syndrome, with possibility to learn new praxies. The intellectual quotient was not altered and associative functions, depending of the posterior third of the corpus callosum were spared. Anti-epileptic medications were not stopped. We think that the improvement of the frontal syndrome is due to reduction of seizures. Therefore, we insist on the interest of the section of the anterior two thirds of the corpus callosum as treatment of tonic and atonic seizures but also as treatment of psychiatric symptoms depending of a frontal syndrome.

Sphenoidal electrode insertion under local analgesia in children.

The indications for method and the results of sphenoidal electrode insertion under local analgesia are evaluated in children. This technique makes it possible to study the hippocampal area, which cannot be studied by other extracranial electrodes. It also localizes in a temporal lobe some complex seizures without electrical events on surface recordings, complex seizures with bilateral temporal spikes or a frontotemporal focus of spikes, as well as those with a temporal focus with bilateral synchronous spikes in standard EEG. Therefore, sphenoidal electrodes inserted without heavy general analgesia enable temporal seizures to be identified and localized, leading to more specific neuroradiological and neurophysiological explorations and helping in this way to select possible patients for epileptic surgery.

[Dopa-sensitive muscular dystonia. Segawa's syndrome. A case report]. / Dystonie musculaire dopa-sensible. Syndrome de Segawa. A propos d'un cas.

A four-year-old girl developed difficulty in walking due to dystonia of the right then left foot, rest tremor in both hands, and rigidity. These symptoms worsened upon exertion and in the evening and were remarkably responsive to L. dopa, suggesting the diagnosis of fluctuating muscular dystonia or Segawa syndrome.

[Mental development in Duchenne muscular dystrophy. Correlation of data of the brain scanner]. / Le développement mental dans la dystrophie musculaire de Duchenne. Corrélation avec les données du scanner cérébral.

In an investigation of 15 patients with Duchenne muscular dystrophy, the authors found mental retardation in all cases. In addition, CT scan showed a cortical atrophy after the age of 10 years. This finding suggests that a cerebral degenerative process is present in cases of Duchenne muscular dystrophy.

[Difficulties in diagnosis of frontal syndrome in epileptic children: clinical, diagnostic, physiopathological aspects. Apropos of 6 cases]. / Difficulté du diagnostic de syndrome frontal chez l'enfant épileptique: aspects cliniques, diagnostiques et physiopathologiques. A propos de six cas.

The authors report 6 cases of acute frontal syndrome following severe seizures of frontal origin. The study of the 6 cases shows the place of disorders in affectivity, behavior, judgement and motor activity; such features changing over time. The relationship between the frontal syndrome and epilepsy is suggested by the fact that the frontal syndrome appears after an increased frequency of frontal seizures, with prolonged discharges of generalized or frontal spikes. The frontal syndrome disappears slowly with the epileptic discharges, and no frontal lesion is found on CT-Scan. Such cases suggest that the frontal syndrome is functional, linked to the localization of the epileptic discharges; it may be regarded as a post-critic deficit, and must be differentiated from a post-critic delirium or a psychotic state.

Migraine in patients with history of centro-temporal epilepsy in childhood: a Hm-PAO SPECT study.

The authors report the results of a retrospective controlled study on the incidence of migraine in centro-temporal epilepsy compared to absence epilepsy, partial epilepsy and a group of patients with cranial trauma without epilepsy. The following observations from this series of 129 patients were made. Migraine was present in 63% of the patients with centro-temporal epilepsy (rolandic epilepsy), in 33% with absence epilepsy, in 7% with partial epilepsy and in 9% of the cranial trauma group. These results suggest that the association of centro-temporal epilepsy and migraine is non-fortuitous and also to a lesser degree in absence epilepsy. The role of neurotransmitters in this association is discussed. No decrease in cerebral blood flow was observed in 12 patients with rolandic epilepsy on a Hm-PAO SPECT study.

[Identification of temporal epilepsy in children: contribution of sphenoidal electrodes]. / L'identification des crises épileptiques temporales chez l'enfant: apport des électrodes sphénoïdales.

The authors have evaluated the indications, method and results of utilising sphenoidal electrodes in epilepsy. This method makes it possible to study the hippocampal area which cannot be studied by extra-cranial electrodes. It also localizes in the temporal lobe complex seizures without investigation by an electroencephalogram, or with a non-specific electroencephalographic picture. Thus, sphenoidal electrodes enable temporal seizures to be identified and localized, leading to more specific neuroradiological exploration, and therefore to the selection of patients for specific surgery.

[Multiple sclerosis in children. Impact of memory disorders]. / La sclérose en plaques chez l'enfant. L'impact des troubles de la mémoire.

Three cases of multiple sclerosis, characterized by disorders of superior functions such as memory loss, either isolated (1 case) or as part of a frontal syndrome (2 cases) are reported. The importance of these disorders, often presenting as the main symptoms, their good prognosis and their rarity in adulthood are underlined. From a pathophysiologic point of view, the authors discuss the role of the frontal, near the limbic area, localization of the plaques.