Surgery in severe factor XIII deficiency: report of a case of epilepsy neurosurgery and review.

Factor XIII (FXIII) deficiency is a rare autosomal recessive congenital disorder of haemostasis, associated with a high risk of intracranial haemorrhage. Intracranial haemorrhage can result in neurological sequelae including seizure disorders. In some cases, medically intractable epilepsy led to epilepsy surgery. Little has been reported on the management of FXIII deficiency during surgery, and there is only a few data on the management, safety and efficacy of epilepsy surgery in the patients with haemostatic disorder. We report here an epilepsy neurosurgery in a case of severe FXIII deficiency.

[Characteristics of the global population]. / Caratteristiche della popolazione globale analizzata.

We analyse clinical characteristics, presurgical investigations, surgical procedures and outcome of 137 patients operated-on for a drug-resistant partial epilepsy, in Grenoble from January 1990 to December 1993. Moreover we present data of 63 patients suffering from a "pure" temporal lobe epilepsy selected using the following criteria: 1. surgery limited to temporal lobe structures 2. totally cured after surgery (Engel's class la).

Chronic trichloroethylene intoxication: a new approach by trigeminal-evoked potentials?

Trigeminal nerve impairment is frequently seen in chronic trichlorethylene intoxication (TRI). A total of 104 occupationally exposed subjects were selected for study because they were employed at a highly exposed workplace. They were studied by clinical examination and by trigeminal somatosensory evoked potentials (TSEP). Normal values for TSEP were obtained from the study of 52 healthy nonexposed subjects. Facial hypoesthesia, when present, was global and predominant in the mandibular and maxillary nerve areas, associated or not with absent reflexes. A disturbed TSEP was found in 40 subjects which was predictable from their clinical symptoms. Correlation with exposure parameters (duration of exposure, trichlorethanol, and trichloracetic acid urinary rates) was mainly observed in subjects presenting both clinical and electrical alterations. Electrical alterations alone were less predictive. An abnormal TSEP may thus provide objective determination of risk assessment in the absence of clinical signs.

[Somatosensory evoked potentials of the trigeminal nerve in chronic trichloroethylene poisoning]. / Potentiels évoqués somesthésiques du trijumeau dans les intoxications chroniques par le trichloréthylène.

Industrial intoxication by trichloroethylene can produce neurological disturbance of the trigeminal nerve. Therefore, among 105 workers exposed to this toxin, we recorded cortical responses after stimulation of the trigeminal nerve (TSEP) and we compared our results with a control group of 52 subjects. We considered the physical examination exploring, the facial sensitivity and reflexes, the levels of urinary metabolites of trichloroethylene and TSEP. We can describe 4 groups of workers: group A, 13 subjects with perturbations of clinical and evoked responses; group B, 27 subjects with normal physical examination though with disturbed TSEP; group C, 6 subjects had an abnormal examination though evoked responses were normal; group D, 59 subjects had normal clinical examination and TSEP. Disturbed TSEP (particularly delayed responses) are found among the older workers with the longest duration of exposure and higher level of exposure. The alteration of TSEP may appear before clinical disturbance. TSEP could represent a useful test for the supervision of the exposed workers and the clinical diagnosis of the trigeminal impairment.

[Variability of V waves of auditory evoked potentials in patients with supratentorial transverse displacement of the brain caused by intracranial hematoma]. / Zmiennosc fali V sluchowych potencjalów wywolanych u chorych z nadnamiotowym prezemieszczeniem poprzecznym mózgu wywolanym krwiakiem sródmózgowym.

The authors present a comparative analysis of the characteristics of the V wave of the brain stem auditory evoked potentials in relation to the degree of transverse shifting of the third ventricle. The investigations were carried out in 48 patients with non-traumatic intracerebral haematoma. A high correlation was found between the size of the amplitude and ventricle shift. No such correlation was observed with the interpeak time of the I--V waves.

[Changes in the semeiology of epileptic seizures after status epilepticus: apropos of 65 cases]. / Modifications de la sémiologie des crises épileptiques après état de mal: à propos de 65 observations.

In 65 patients with status epilepticus, we compared the clinical expression of isolated seizures and of status seizures. In 22 patients there was no relationship between status and isolated seizures. In addition the number of partial status is greater than that of partial seizures. In 9 patients, the type of seizures was modified after the status. From these results, status epilepticus seems to favor the eruption of secondary epileptogenic focuses, generally transitory.

[Brain stem AEP in toxic comas. Correlation with brain stem reflexes]. / Etude des PEA du tronc cérébral dans les comas toxiques. Corrélations avec les réflexes du tronc cérébral.

24 deep toxic comas with respiratory assistance were studied by brain-stem reflex and BAEP. Laboratory analysis showed several simultaneous toxics (phenobarbital, benzodiazepines, tricyclic antidepressants...). Three groups of patients were defined: Twelve patients with normal BAEP and with relatively preserved brain-stem reflex (oculocephalic and oculovestibular are often disturbed but photomotor is present). Eleven patients with delayed BAEP and with more disturbed brain-stem reflex (photomotor is missing 3 times). In 2 cases (one of which is mentioned above in group B) brain-stem reflex and BAEP disappear and these patients die. Cerebral anoxia is associated here with toxics. Causes of delayed latencies (group B) are discussed (hypothermia, toxics). BAEP seems important in diagnosis and prognosis of toxic coma.

[Seizures in the newborn infant; value of polygraphy]. / Les crises du nouveau-né; intérêt de la polygraphie.

Recordings were made from 20 newborns during seizures in their first days of life, using a polygraphic study enabling observation of electro-clinical seizures, electric seizures, and seizures with no EEG transfer. The interictal EEG as well as the duration of status epilepticus are discussed. An estimation of the prognosis from the EEG clinical criteria is envisaged. CT scans and ultrasound scans were performed in serious cases of neonatal distress responsible for status epilepticus; these showed diffused or localized oedema of the white matter, either isolated or associated with intracranial hemorrhagic lesions.

Evoked trigeminal nerve potential in chronic trichloroethylene intoxication.

Results of a study of trigeminal nerve impairment resulting from trichloroethylene intoxication by the somatosensory-evoked potential method reveal three kinds of abnormalities: increased stimulation voltage, excessive latency delay with morphological abnormalities, and excessive graph amplitude. These abnormalities confirm clinical disturbance (hypesthesia of the trigeminal nerve area) and open debate about the real mechanism of trichloroethylene neurotoxicity. Industrial intoxication by solvents, particularly trichloroethylene, is common. We have conducted a study of 188 workers chronically exposed to trichloroethylene and have confirmed the selective neurological disturbances of this intoxication in the trigeminal nerve (20%) [3, 10]. We utilized a new experimental method, developed for studies of chronic intoxications effecting the median nerve [5, 8], of recording the somatosensory evoked potential following stimulation of the trigeminal nerve [4, 6, 7]. The workers in this study were selected following clinical evaluation of their facial sensitivity and trigeminal nerve reflexes. In this paper we present our preliminary results on 11 workers, 9 suffering effects of intoxication and 2 controls.

[Value of the study of trigeminal nerve evoked potentials in chronic trichloroethylene poisoning. Preliminary results]. / Intérêt de l'étude des potentiels évoqués du trijumeau dans l'intoxication chronique au trichloréthylène. Résultats préliminaires.

Results of a study concerning the trigeminal nerve impairment as the one caused by trichlorethylene chronic intoxication by somatosensory evoked potential reveal three kinds of anomalies: increase of the stimulation voltage, excessive latency delay with morphological anomalies and excessive graph amplitude. These anomalies confirm clinical disturbance (hypoesthesia in the trigeminal area) and open a debate about the real mechanism of the trichlorethylene neurotoxicity.

[EEG and clinical study of 107 patients with acute severe traumatic comas (author's transl)]. / Surveillance électroclinique de 107 comas traumatiques graves aigus.

The authors describe the EEG recordings of 107 patients with acute severe cranial trauma and signs of axial lesions. The initial EEG included: sleep patterns, alternating tracings, alpha band tracings which were either nonreactive pseudo-alpha or modulated alpha often reactive, and diffuse slow wave tracings. They found no particular tracing corresponding to a precise level of rostrocaudal destructuration, but some associations were seen more frequently. EEG sleep patterns and alternating tracings were never observed in brain stem lesions, the activity recorded being of low voltage, rigid, and non-reactive. The presence of spontaneous fluctuations (with sleep rhythms in some cases) and reactivity in other levels (mesodiencephalic junction, diencephalic and cortical-subcortical) are good prognostic signs. Focalised EEG signs are seen especially in the more rostral levels.

[Mesodiencephalic level of severe head injuries (author's transl)]. / Le stade méso-diencéphalique des traumatismes craniens graves.

From the study of 65 severe traumatic comas, the authors describe the syndrome of mesodiencephalic dysfunction, because of its frequency and its particular clinical signs. This peculiar level of loss of consciousness is characterized by a coma without mimic and without awakening after painful stimulation, by a stereotyped motor pattern in extension, by a perseverance of the photomotor reflex, while fronto-orbicular and vertical oculo-vestibular reflexes are lacking. This level of deterioration appears to be a critical point in the natural history of comatose patients. If this level is not surpassed, the probability of early death due to evolutive neurological lesion is low (18%). One half of these patients have a good prognosis, more than 75% have an excellent recovery. This high probability of recovery is decreased by a half when the level of mesencephalic dysfunction is reached.

[Myoclonic type of Huntington's chorea (author's transl)]. / Formes myocloniques de la chorée de Huntington.

The authors report the case of an 8 year-old boy who, when he was 2 1/2 years old, suffered from spasmodic mouth twitches. At the age of four, various other symptoms appeared: psychomotor backwardness, frequent fails and a photomyoclonic response on electroencephalogram. At the age of 5 1/2, noticeable difficulties appeared in walking with a broad-based gait, hypotonia, and intentional trembling associated with hypokinesia and dysarthria. When he was six, the first convulsive seizure appeared, then myoclonies which became continuous. The child gradually became bedridden. The family history tends to show these disorders can be linked with a Huntington chorea affecting six generations. This case is very similar to that previously described by the authors, in an 8 year-old girl where an anatomic examination revealed the existence of lesions characteristic of Huntington's disease associated with lesions of the cerebellum. The authors, on the basis of the data provided by the literature, discuss the myoclonic and cerebellous aspect of this infantile form. Lacking anatomic evidence, they stress the interest of the biochemical disturbances affecting the cerebral monoamines noted in this observation.

[Reye's syndrome a clinical and electrophysiological study of four patients (author's transl)]. / Le syndrome de reye étude électroclinique de quatre observations.

The authors studied four children with Reye's syndrome aged 16 months, 6 years, 8 years and 11 years respectively with severe liver failure and progressive coma. The laboratory investigations in all of them showed a marked elevation of serum transaminases, hyperammoniaemia and a prolongation of the prothrombin time. The electroencephalograms showed a grossly abnormal picture with generalised continuous delta activity. Three children survived with return to normal of the liver function tests whilst the fourth child died. In the last two patients an electroencephalogram carried out every six hours has enabled certain prognostic features to be determined. Improvement in the E.E.G. correlates closely with clinical improvement and vice versa. The authors also advocate serial E.E.G. recordings in Reye's syndrome. The role of hyperammonaemia in the genesis of encephalopathy and the electroencephalographic changed is discussed. The role played by raised intracranial pressure is stressed and the importance of controlling it in order to prevent further damage and improve the prognosis of this serious illness.