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Thirteen parkinsonians with a long duration of the disease and longterm dopa therapy, seven of them showing severe on-off oscillations and 6 an "endof-dose impairment", were treated with a controlled release (HBS) preparation of L DOPA/benserazide for more than 3 years.Thereafter, selegiline was added in a progressively increasing dosage up to a maximum of 10 mg/day during 4 months, with the aim of a) further improving the longterm results and b) reducing the doses of the new formula of LDOPA.A significant decrease of early morning parkinsonism and reduction of motor disability throughout the day were observed; "wearing-off" cases showed better results compared with those presenting "on-off" oscillations. A mean reduction of 20 ' 70 in the doses of levodopa was achieved. Likewise, a mild reduction of dyskinesias and a mild-moderate enhancement of dystonias were recorded. Only one patient did not tolerate selegiline and two others received lower doses due to side-effects.Selegiline was capable of enhancing the antiparkinsonian effect of the new formula of LDOPA, while allowing a reduction of the doses administered. It must also be emphasized that such improvement was achieved in complicated patients, most of whom showed some deterioration of response in the late stages of long-term sustained-release levodopa treatment.

Fourteen parkinsonian patients, 10 of them showing severe and long-standing ‘on-off effects and 4 ‘end-of-dose impairment’, received Madopar HBS instead of standard Madopar. At the end of the dosage adaptation phase (9 weeks) most patients improved; in patients with ‘on-off phenomenon, parkinsonism became less severe, on periods were longer, and fluctuations decreased; end-of-dose impairment resolved in 4 patients. However, a longer delay in the onset of the therapeutic effect was observed after the first daily drug intake in those patients still showing severe early-morning parkinsonism. With Madopar HBS, L-dopa dosage was increased by 116 %. In spite of a greater dopaminergic effect, dyskinesias were reduced, and dystonias became less marked or even disappeared.

Four members of a family with consanguineous relationships, the proband and his three children (2 sons and 1 daughter) are affected with Familial Spastic Ataxia and with Ehlers-Danlos' Syndrome with platelet aggregation dysfunction. In the four cases, this exceptional association appears remarkably homogeneous both in clinical and laboratory studies. The two syndromes are of dominant-autosomic transmission and probably originated in a new mutation which presumably maintained a genetic linkage. Spastic ataxia is characterized by a precocious onset and a slow evolution. The first-born son shows a dominant pyramidal syndrome with mild ataxia suggesting that it is a transitional form of familial spastic paraplegia. The Ehlers-Danlos syndrome pertains to form II or "mitis" with moderate skin hyperelasticity and joint hypermobility. The abnormal platelet aggregation curves have the same profile in all the patients. The first-born son also presents a mitral valve prolapsus as we may find either in Ehlers-Danlos syndrome or in spastic ataxia.The neurophysiological, tomographical, histological, ultrastructural and biochemical studies attempt to accomplish a better definition of these associated nosological entities. RESUME: Dans une famille avec des rapports de consanguinite, quatre de ses membres, le "proband" et ses trois enfants (deux hommes et une femme) sont atteints d'une ataxie spastique et du syndrome d'Ehlers-Danlos avec deficit d'agrdgation des plaquettes. Cette extraordinaire association montre des aspects cliniques et de laboratoire tres homogenes dans les quatre cas. Les deux syndromes se transmettent d'une maniere autosomale dominante et originent probablement dans une nouvelle mutation qui maintient peut-etre un lien gfinetique. L'ataxie spastique se caracterise par un debut tres pr6coce et une lente Evolution. L'aine montre un syndrome pyramidal dominant avec une 16gere ataxie, ce qui suggere une forme transitionnelle avec la paraplegie spastique familiale. Le syndrome d'Ehlers-Danlos correspond a la forme II ou "mitis" avec une hyperelasticity moderee de la peau et une hypermobilite articulaire. Les courbes anormales d'agregation des plaquettes ont le meme profit chez tous les patients. L'aine' montre un prolapsus de la valve mitrale tel que nous pouvons le trouver dans le syndrome d' Ehlers-Danlos ainsi que dans l'ataxie spastique. Les dtudes neurophysiologiques, tomodensitometriques, histologiques, ultrastructurelles et biochimiques essaient de deTmir le mieux possible cette association d'entites nosologiques.

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