[Treatment of central and neuropathic facial pain by chronic stimulation of the motor cortex: value of neuronavigation guidance systems for the localization of the motor cortex]. / Traitement des douleurs centrales et neuropathiques faciales par stimulation chronique du cortex moteur. Intêret des systèmes de neuronavigation pour le repérage du cortex moteur]

Thirty two patients with refractory central and neuropathic pain of peripheral origin were treated by chronic stimulation of the motor cortex between May 1993 and January 1997. The mean follow-up was 27. 3 months. The first 24 patients were operated according to the technique described by Tsubokawa. The last 13 cases (8 new patients and 5 reinterventions) were operated by a technique including localization by superficial CT reconstruction of the central region and neuronavigator guidance. The position of the central sulcus was confirmed by the use of intraoperative somatosensory evoked potentials. The somatotopic organisation of the motor cortex was established peroperatively by studying the motor responses at stimulation of the motor cortex through the dura. Ten of the 13 patients with central pain (77%) and nine of the 12 patients with neuropathic facial pain had experienced substantial pain relief (75%). One of the 3 patients with post-paraplegia pain was clearly improved. A satisfactory result was obtained in one patient with pain related to plexus avulsion and in one patient with pain related to intercostal herpes zoster. None of the patients developed epileptic seizures. The position of the stimulating poles effective on pain corresponded to the somatotopic representation of the motor cortex. The neuronavigator localization and guidance technique proved to be most useful identifying the appropriate portion of the motor gyrus. It also allowed the establishment of reliable correlations between electrophysiological-clinical and anatomical data which may be used to improve the clinical results and possibly to extend the indications of this technique.

Motor cortex stimulation in the treatment of central and neuropathic pain.

BACKGROUND: Motor cortex stimulation has been proposed for the treatment of central pain. METHODS: Thirty-two patients with refractory central and neuropathic pain of peripheral origin were treated by chronic stimulation of the motor cortex between May 1993 and January 1997. The mean follow-up was 27.3 months. The first 24 patients were operated on according to the technique described by Tsubokawa. The last 13 cases (8 new patients and 5 reinterventions) were operated on by a technique including localization by superficial CT reconstruction of the central region and neuronavigator guidance. The position of the central sulcus was confirmed by the use of intraoperative somatosensory evoked potentials. The somatotopic organization of the motor cortex was established preoperatively by studying the motor responses at stimulation of the motor cortex through the dura. RESULTS: Ten of the 13 patients with central pain (77%) and 10 of the 12 patients with neuropathic facial pain experienced substantial pain relief (83.3%). One of the three patients with post-paraplegia pain was clearly improved. A satisfactory result was obtained in one patient with pain related to plexus avulsion and in one patient with pain related to intercostal herpes zoster. None of the patients developed epileptic seizures. CONCLUSIONS: Our results confirm that chronic stimulation of the motor cortex is an effective method in treating certain forms of refractory pain.

Marked systemic amyloid angiopathy in patients with val 107 transthyretin mutation.

We report three non-inbred patients with Val 107 transthyretin (TTR) amyloidosis. Clinical features were remarkable by the combination of peripheral polyneuropathy, carpal tunnel syndrome, cardiomyopathy, and epilepsy. Pathologic examination disclosed unusual striking systemic amyloid angiopathy in all studied tissues including nerve, muscle, gut, lung, salivary glands, and synovial membrane. It appears that the rare TTR Val 107 variant causes a peculiar familial amyloid syndrome characterized by both widespread systemic TTR amyloidosis and central nervous system deposition sufficient to cause seizures, pointing out the extent of TTR amyloidosis phenotypic heterogeneity.

T cell subsets in multiple sclerosis. A longitudinal study of exacerbating-remitting cases.

Twenty-four untreated MS patients with exacerbating-remitting disease were longitudinally studied for T-cell subset distribution within peripheral blood, using monoclonal antibodies OKT3, OKT4 and OKT8. A decreased percentage of OKT8 reactive cells, with a correlative increase of the OKT4/OKT8 ratio, was detected in relapsing MS patients, in most cases within 2 weeks before and 1 week after the onset of relapse. Longitudinal analysis of individual fluctuations allowed to detect during relapse an increase of OKT4/OKT8 ratio over the value recorded during remission in 78% of MS patients. Only 50% of patients however exhibited an OKT4/OKT8 ratio exceeding the 5% confidence upper limit of healthy control values. Relapse was more often associated with T-cell subset abnormalities in patients who suffered several attacks during the period of study. MS patients in remission, when considered as a group did not show significant abnormalities of the T-cell subset balance, although some individuals did present with wider T-cell subset fluctuations than healthy controls.

Luyso-pallido-nigral atrophy and amyotrophic lateral sclerosis.

The clinical and pathologic findings in a 34-year-old woman with basal ganglia degeneration and amyotrophic lateral sclerosis are reported. The duration of symptoms was 2 years. A maternal uncle had a parkinsonian syndrome with onset at 45 years of age. Neuropathologic examination revealed extensive neuronal loss and gliosis in the corpus Luysii. Nerve cell loss and gliosis also involved both parts of the globus pallidus, and the substantia nigra. The corticospinal tracts were demyelinated in the spinal cord, and neuronal loss was observed in the anterior horns. Only one similar case of pallido-luyso-nigral atrophy associated with amyotrophic lateral sclerosis has, to our knowledge, been reported previously. Such an association may represent more than a coincidental occurrence.

Adult form of Leigh's disease: a clinico pathological case with CT scan examination.

The clinical and pathological findings of a 31-year-old woman, in whom the diagnosis of Leigh's disease was made, are reported. CT scan examination with contrast enhancement showed symmetrical areas of low density, in both thalami, anterior limbs of internal capsules and corpus callosum. Longstanding chronic lesions involved the optic chiasma and the cerebral peduncles and consisted of myelin loss, status spongiosus, astrocytic gliosis and marked capillary proliferation. The neurons were spared. In the basal ganglia, internal capsules and corpus callosum, these lesions were more recent and consisted of focal necrosis, perivascular oedema and few lymphocytic perivascular cuffings.

[Cerebral x-ray computed tomography and tuberculous meningitis. Radio-clinical correlations]. / Scanographie cérébrale et méningite tuberculeuse. Corrélations radio-cliniques.

Two cases of tuberculous meningitis showed that there is a delay between the C.T. images and the clinical signs. The computed tomography which was still normal one and two months after the first clinical signs did not permit an early diagnosis of the tuberculous meningitis. It did not give either, at least in the beginning, a good appreciation of the results of the treatment, since most of the C.T. images appeared and/or increased while the patient's conditions had already been improving under treatment for several days or weeks. In a long range, however, the C.T. images usually disappear.

Monoclonal antibody-defined immunoregulatory cells in multiple sclerosis cerebrospinal fluid.

To determine if previously reported peripheral blood suppressor cell defects are also found in the central nervous system (CNS) of patients with multiple sclerosis (MS), we studied cerebrospinal fluid (CSF) and peripheral blood lymphocytes from 40 MS patients and 15 patients with other neurological diseases. With an indirect immunofluorescence technique using the OKT series of monoclonal antibodies (OKT4, marking helper/inducer cells, OKT5 and OKT8 marking suppressor/cytotoxic cells, and OKT3 marking all peripheral T cells) we found that MS patients tested in the first 2 wk of exacerbation had invariably diminished CSF suppressor/cytotoxic cells, which was followed by an elevation of these cells in the 3rd wk of exacerbation. Repeat studies of three patients showed that perturbations of CSF suppressor/cytotoxic cells were dependent on clinical status. These observations add to the accumulating data that suggest altered immunity in the pathogenesis of MS.

Serum and cerebrospinal fluid C2 in multiple sclerosis.

C2 hemolytic activity was quantitated in the serum and cerebrospinal fluid of 46 MS patients studied twice at a 1-year interval. whereas serum C2 levels were found within the normal range, CSF C2 values were decreased (55 and 59% of normal) in patients with severe active disease. In contrast CSF C2 levels were normal in patients with stable or recently improved condition. CSF C2 fluctuations in individuals were found to closely parallel changes in the clinical course.

[Myopathy, osteomalacia, and congenital hypoalbuminemia (author's transl)]. / Myopathie. Ostéomalacie et hypoalbuminémie congénitale.

A myopathy revealed the presence of a vitamin-sensitive osteomalacia in a patient with congenital hypoalbuminemia. The severe myopathy and osteomalacia recovered after treatment with 25-hydroxycholecalciferol. Hypoalbuminemia was detected in two brothers of the patient; parents and grandparents were consanguineous. Various tests demonstrated that the patient's hypoalbuminemia was due to faulty synthesis. Albumin reactions to several drugs were normal, but those normally transported by albumin have to be administered cautiously because of the low plasma concentrations. The possible relationship between hypovitaminosis D and congenital hypoalbuminemia, an association considered to be fortuitous by Montgomery, remains debatable.

[Probable cerebral tuberculoma in an apparently healthy Caucasian: normal computed tomography recordings after two months of treatment (author's transl)]. / Tuberculome cérébral probable chez un métropolitain floride: normalisation tomodensitométrique en deux mois de traitement.

A probable case of cerebral tuberculoma is described. An atypical feature was the occurence of the lesion in an apparently healthy Caucasian. Computed tomography examinations enabled the course of the disease to be followed-up during treatment.