Subject(s)
Essential Tremor/etiology , Hyperthyroidism/diagnosis , Parkinson Disease/diagnosis , Parkinsonian Disorders/etiology , Tremor/etiology , Aged , Antiparkinson Agents/therapeutic use , Antithyroid Agents/therapeutic use , Combined Modality Therapy , Deep Brain Stimulation , Diagnosis, Differential , Essential Tremor/therapy , Female , Humans , Hyperthyroidism/complications , Hyperthyroidism/drug therapy , Levodopa/therapeutic use , Neurologic Examination , Parkinson Disease/therapy , Parkinsonian Disorders/therapy , Tremor/therapy , Ventral Thalamic Nuclei/physiopathologyABSTRACT
Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.
Subject(s)
Fibroma/pathology , Fibromatosis, Gingival/pathology , Skin Neoplasms/pathology , Biopsy , Contracture/pathology , Facies , Humans , Infant , Male , Microscopy, ElectronSubject(s)
Antiparkinson Agents/adverse effects , Electric Stimulation Therapy , Levodopa/adverse effects , Movement Disorders/etiology , Movement Disorders/prevention & control , Parkinson Disease/drug therapy , Aged , Electrodes, Implanted , Humans , Male , Thalamic Nuclei/surgery , Thalamus/surgeryABSTRACT
We studied the effect of intracerebroventricular infusion of dopamine and dopamine agonists in rat and primate models of Parkinson's disease as an experimental approach to the treatment of levodopa-induced fluctuations. The infusion of dopamine, lisuride, and pergolide into the ventricle ipsilateral to the lesion, by 6-hydroxydopamine, of the nigrostriatal pathway induced a contralateral rotation which was maximal 24-48 h after infusion and whose intensity progressively decreased over the period of 1 week. [3H]Spiperone binding was decreased by the infusion of dopamine but the responses to subcutaneous apomomorphine were unchanged. The infusion of dopamine also restored the levels of monoamines in the rat brain. In chronic reserpized rats, the infusion of dopamine restored brain levels of dopamine but did not reverse akinesia unless monoamine oxidase inhibitors were simultaneously administered, either systemically or intracerebroventricularly. Lisuride and pergolide proved much weaker than dopamine in reversing the effects of reserpine. Intracerebroventricular infusion of dopamine plus deprenyl reversed MPTP induced akinesia in monkeys but the pump used for the delivery was not well tolerated, because of its size, by the animals.
