Cranial pachymeningitis of unknown origin: a study of seven cases.

We report seven patients with cranial pachymeningitis of unknown origin in whom the main clinical features were headaches, ataxia, and cranial nerve palsies. CSF showed inflammatory changes. CT and MRI showed thickening of the falx and of the tentorium. The clinical course was chronic. Four patients improved with prednisolone but became steroid-dependent: in two cases, radiotherapy had no lasting improvement and in one, azathioprine permitted a reduction of the corticosteroids. Five patients had biopsy of the tentorium cerebelli or of the temporal dura mater. In two cases, autopsy revealed extensive pachymeningitis without parenchymal changes. In all instances, microscopic examination of the dura mater showed a cellular infiltrate of polymorphic cells; there were no epithelioid granulomas. Review of the literature discloses seven similar cases. We discuss the relationship of these lesions with inflammatory meningeal masses, the focal pachymeningitis of the Tolosa-Hunt syndrome, and multifocal fibrosis.

[Slowly progressive motor neuron disease associated with paralysis of the abductor muscles of the vocal cords]. / Atteinte motrice périphérique lentement progressive associée à une paralysie des muscles abducteurs des cordes vocales.

Some cases of slowly progressive motor neuron disease with bilateral paralysis of the vocal cord abductor muscles have recently been published. We report a case with a more than forty year's course occurring in a family in which one brother and probably 2 cousins died of motor neuron disease.

[Clinical recovery from Isaacs' syndrome]. / Guérison clinique du syndrome d'Isaacs.

A patient with Isaacs' syndrome (previously reported in 1966) has been clinically normal since 1972. The possibility of an outcome of this type has been reported by Isaacs and Heffron (1974) and in subsequent reports. This does not provide an explanation for the syndrome of continuous muscle fiber activity but constitutes an additional clinical feature.

[Supratentorial glioblastoma in adults. Therapeutic results]. / Glioblastomes sus-tentoriels de l'adulte. Résultats thérapeutiques.

This retrospective study concerns 55 adult patients with supratentorial glioblastoma. The tumours were treated by complete or partial surgical excision whenever possible (31 cases), radiotherapy (22 cases) in doses of 60 Gy over 6 to 7 weeks (40 Gy with telecobalt and 20 Gy with superimposed electrons) and multiple chemotherapy (10 cases) with VM26 and CCNU or BCNU. Although the number of patients in some categories was too small for statistical evaluation, the results obtained were in agreement with those found in the literature and indicative of what can be expected. In patients with inoperable tumours the mean survival was increased from 2 to 8 months by radiotherapy or chemotherapy given separately, and from 2 to 9 or 10 months only when these two methods were combined. The mean survival of patients with partial tumoral excision was 2.5 months extended to 10 months after post-operative radiotherapy; one patient in this group who received both radiotherapy and chemotherapy is still alive after more than 5 years. In patients with macroscopically satisfactory excision, the 12 months good quality survival obtained by surgery was apparently prolonged to 22 months with radiotherapy; 3 of these patients who had both radiotherapy and chemotherapy after surgery are alive and in good condition after 15, 16 and 28 months respectively.

[The Marcus Gunn phenomenon]. / Le phénomène de Marcus Gunn.

Ten cases of the Marcus Gunn phenomenon are reported. This synkinesis presents as an involuntary raising of the upper eyelid, often exhibiting ptosis at rest, when the lower jaw drops or deducts towards the opposite side. Hypotheses proposed to explain its mechanisms are reviewed, and the differential diagnosis is discussed in relation to the Marin Amat syndrome, the significance of which is considered. In addition, a case of rarely reported faciopalpebral synkinesis is also reported, the patient presenting involuntary raising of an upper eyelid during spontaneous or voluntary smiles.

[Creutzfeldt-Jakob disease of 34 month's development in a 26-year-old woman]. / Maladie de Creutzfeldt-Jakob ayant évolué 34 mois chez une femme de 26 ans.

A clinico-pathological case of C. J. disease which occured in a 26 year old patient and evolved over 34 months is reported. Published cases of patients dead before 30 y.o. are briefly reviewed. The average length of evolution was 28 months. Concerning the french cases it appears that this average length was significantly longer when C. J. disease had started before 50 y.o., than when it had begun after 75 y.o. However the general survey of the published cases does not show any correlation between the starting age of the disease and the duration of evolution.

[Cerebral cystercosis. 3. cases observed in the Paris area in immigrant workers]. / Cysticercose cérébrale. Trois cas observés dans la région parisienne chez des travailleurs immigrés

The three cases described involve two Portugese patients and a Spaniard working in the Paris are who had presented with epilepsy, being focal in two. Diagnosis was made in two cases as a result of the existence of muscular and sub-cutaneous calcifications; in one case because of eosinophilia of the cerebro-spinal fluid. The clinical and therapeutic features of cerebral cysticercosis are recalled briefly in connection with these cases.