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Biomedical subjects

S Fahn

Publications and source records attributed to S Fahn.

At least 217 records · Page 12Linked to original sources
PubMed1988

An estimate of the prevalence of dementia in idiopathic Parkinson's disease.

A review of the records for evidence of dementia using criteria adapted from the third edition of the Diagnostic and Statistical Manual of Mental Disorders in every patient (hospitalized and outpatient) with parkinsonism at a major medical center during an 18-month period revealed an overall prevalence of 10.9% in 339 patients with idiopathic Parkinson's disease. Demented patients were older, had a later age at onset of motor manifestations, and a more rapid progression of physical disability than nondemented patients. Duration of illness and levodopa use and the presence of tremor or depression were similar in demented and nondemented patients. Demented patients more often responded poorly or developed adverse effects to levodopa than nondemented patients. When Parkinson's disease began after age 70 years, dementia was noted over three times more frequently than when the disease began at an earlier age. The age-specific prevalence rate of dementia for patients older than 70 years was more than twice that for younger patients. Moreover, the number of records with evidence for dementia with idiopathic Parkinson's disease was 3.75 times greater than expected in comparison with data from a study of the prevalence of dementia in the elderly.

Aged↗
PubMed1988

Analysis of open-label trials in torsion dystonia using high dosages of anticholinergics and other drugs.

We reviewed the records of 358 patients with various forms of focal, segmental, and generalized dystonia who had received pharmacotherapy in a systematic order, beginning with anticholinergics. If no benefit was encountered, we then tested clonazepam, baclofen, and other agents sequentially. In each situation the dosage was gradually increased until benefit or troublesome adverse effects were encountered. In this manner we obtained data on the percentage of patients who showed moderate to marked benefit. Anticholinergics were the most beneficial agent, confirming previous reports. Statistical analysis of this response revealed that benefit from anticholinergics was most likely if treatment was begun within 5 years after the onset of dystonia. From this analysis, it appears that delaying treatment beyond 5 years is likely to result in an unsatisfactory response.

Adolescent↗
PubMed1988

Glycine involvement in DDT-induced myoclonus.

The DDT syndrome in rats consists of tremor, myoclonus, running seizures, hyperthermia, episodic boxing, and excessive grooming. DDT did not change whole-brain glycine levels when the rats had stimulus-sensitive myoclonus, spontaneous myoclonus, or seizures. However, regional analysis showed a decrease in glycine levels in the pons and medulla initially, but they rose again despite worsening of the myoclonus. Glycine given intraventricularly and the glycine prodrug, milacemide, given intraperitoneally suppressed DDT-induced myoclonus. A dose of milacemide that prevented DDT-induced myoclonus caused a significant increase in glycine levels in cortex, septum accumbens, cerebellum, striatum, hippocampus diencephalon, midbrain, pons, and medulla. The increase was most marked in the forebrain structures. There was no change in serine levels in these areas. These data suggest that the glycinergic system may be playing an important role in the manifestation of DDT-induced myoclonus.

Acetamides↗
PubMed1988

Therapeutic trial with glycine in myoclonus.

We investigated the therapeutic effects of glycine in seven patients with various forms of myoclonus. The initial phase was an open label trial. If benefit was seen in any patient, a double-blind substitution of placebo was carried out to determine if the benefit was due, in fact, to glycine. The dosage of glycine was initiated at 600 mg/day and was increased gradually until a maximum dosage of 6,000 mg/day was reached. This dosage was maintained for at least 6 weeks before lack of efficacy was declared. No improvement was seen in four patients. One patient reported improvement, but he discontinued the drug because of adverse effects encountered with the use of a concomitant medication and before he could be tested in a double-blind crossover phase. Two other patients also noticed improvement, but these improvements were not validated in the crossover phase. There were no adverse effects associated with glycine. Plasma glycine levels peaked at 30 min and returned to normal 1.5-h after 1 g of glycine.p.o. Cerebrospinal fluid (CSF) glycine levels did not change during treatment, suggesting inadequate penetration into the central nervous system of glycine at the dosage used.

Adolescent↗
PubMed1988

Neurochemical findings in neuroacanthocytosis.

We performed a neurochemical study of the brain of two unrelated patients, living in different continents, with neuroacanthocytosis. The levels of monoamines and their metabolites, gamma-aminobutyric acid and substance P, were measured in several brain areas and the monoamine metabolites in cerebrospinal fluid. The binding of 3H-spiperone to striatal membranes and to lymphocytes was also measured. Both patients had a progressive neurological disorder with onset in the third decade of life and characterized by a complex movement disorder, epilepsy, muscular wasting, and changes in behavior. The movement disorder initially manifested with oromandibular dystonia and limb chorea, but at the time of death was characterized by a severe dystonic syndrome. The chemical changes were similar in the two patients. The most important neurochemical findings were a depletion of dopamine and its metabolites in most brain areas, most notably in the striatum, and elevation of norepinephrine levels in the putamen and globus pallidus. Substance P was markedly reduced in the striatum and substantia nigra. Our findings may provide clues to the neurochemical mechanisms underlying dystonia.

Acanthocytes↗
PubMed1988

Continuous intracerebroventricular infusion of dopamine and dopamine agonists through a totally implanted drug delivery system in animal models of Parkinson's disease.

We studied the effect of intracerebroventricular infusion of dopamine and dopamine agonists in animal models of dopamine deficiency as an experimental approach to the treatment of levodopa induced fluctuations in Parkinson's disease. Dopamine deficiency was produced in rats by unilateral lesion of the nigrostriatal pathway or by chronic treatment with reserpine. Monkeys were lesioned by intravenous injection of MPTP. The animals were treated with intracerebral infusions of dopamine (with or without associated intraperitoneal administration or intracerebroventricular infusion of pargyline), lisuride and pergolide. The intracerebroventricular infusion of these drugs was performed with osmotic minipumps in rats and with infusaid pumps in the monkeys. The infusion of dopamine or dopamine agonists in rats with unilateral lesions by 6-OH-dopamine produced a persistent rotation contralateral to the lesioned and implanted side. The infusion of dopamine reversed reserpine-induced akinesia only when pargyline was associated. In the range of concentration used, maximum allowed by solubility of compounds, the effects of dopamine were more potent than those of the agonists. In spite of the stability of dopamine "in vitro" when dissolved in antioxidants and at low pH, a pigment, product of autooxidation, was found in the brains of the animals infused with dopamine. The monkeys were implanted with infusaid pumps and infused for up to 3 weeks. The pump was not well tolerated due to its huge size for the animals. One monkey showed reversal of the MPTP-induced akinesia while the other, whose catheter had moved from the correct implantation site, remained unchanged. In both monkeys there was evidence of autooxidation of dopamine. Intracerebral infusion of dopamine agonists may be a possible experimental alternative to the treatment of levodopa induced fluctuations in Parkinson's disease but stable and soluble dopamine agonists and suitable delivery systems are needed.

Animals↗
PubMed1988

Behavioral effects of alpha-benzyl-N-methylphenethylamine (ABNMP), a methamphetamine analog: inhibition by ketanserin and para-chlorophenylalanine (PCPA).

Acute administration of alpha-benzyl-N-methylphenethylamine (ABNMP) induces lateral heavings, straub tail, backward locomotion, and hindlimb abduction-which are all components of the serotonin (5-HT) syndrome. Ketanserin, a 5-HT-2 receptor antagonist and pretreatment with the 5-HT neurotoxin parachlorophenylalanine (PCPA) attenuated the manifestation of these behavioral abnormalities. These results suggest that ABNMP may cause an acute release of 5-HT similar to that elicited by para-chloroamphetamine.

Animals↗
PubMed1988

Clonazepam, haloperidol, and clonidine in tic disorders.

We reviewed the treatment outcomes of 81 patients with multifocal tic disorders followed in our movement disorders clinic. From the three drugs used, rank orders of effectiveness were haloperidol, clonazepam, and clonidine. Because of the risk of tardive dyskinesia, we suggest first a trial with clonazepam, later combined with clonidine if clonazepam alone is not effective. Haloperidol should be reserved for the most disabling cases, and given only after trials with both clonazepam and clonidine have failed.

Child↗
PubMed1988

Effect of high doses of dietary vitamin E on the concentrations of vitamin E in several brain regions, plasma, liver, and adipose tissue of rats.

The object of this study was to assess the influence of high levels of dietary vitamin E on vitamin E concentrations in specific areas of the brain. Four-week-old male rats were fed vitamin E-deficient, control, and high-vitamin E (1,000 IU/kg) diets for 4 months. Concentrations of alpha-tocopherol in serum, adipose tissue, liver, cerebrum, cerebellum, and striatum were determined by liquid chromatography with fluorescence detection. In the high-vitamin E group, alpha-tocopherol concentrations in cerebrum, cerebellum, and striatum increased uniformly to 1.4-fold of values in controls; serum, adipose tissue, and liver attained even higher concentrations: 2.2-, 2.2-, and 4.6-fold, respectively, of control values. As observed before, brain levels of alpha-tocopherol were somewhat resistant to vitamin E deficiency, in contrast to the peripheral tissues.

Adipose Tissue↗
PubMed1988

Localized injections of botulinum toxin for the treatment of focal laryngeal dystonia (spastic dysphonia).

Spastic dysphonia is a condition producing a strain-strangle phonation. We have previously classified most of these patients as having focal laryngeal dystonia, a disorder of central motor processing. The initial success of recurrent nerve section in many of these patients has been followed by recurrence of symptoms in months to years. Bilateral involvement of the vocal cords with hyperfunction of the nonparalyzed vocal cord could explain these failures. Injection of botulinum toxin (BOTOX) has been effective treatment for many focal dystonias. We have treated more than 100 patients with dystonia including five with laryngeal dystonia. All of the patients laryngeal had dramatic improvement after 48 to 72 hours; benefit lasted 3 to 9 months for each injection period. BOTOX injection can be performed on awake, ambulatory patients. Bilateral treatment and titration of dose can achieve the desired degree of weakness.

Adult↗
PubMed1988

Clinical and laboratory characteristics of focal laryngeal dystonia: study of 110 cases.

Spastic dysphonia is a syndrome often producing a strain-strangle voice. We have previously classified most of these patients as having focal laryngeal dystonia, a disorder of central motor processing. In a study of 1,280 cases of dystonia registered at the Dystonia Clinical Research Center at the Columbia-Presbyterian Medical Center, we found 110 patients who had vocal cord involvement. These patients had historical information evaluated for age of onset (mean 34.6 years), duration of symptoms (mean 13.8 years), sex (1.4:1 female to male) family history (positive in 23%), and primary (66%) and secondary (34%) etiology; neurological evaluation for other dystonic involvement (25% with segmental cranial involvement, 23% with generalized dystonia) or tremor (irregular 23%, regular 6% on EMG). Treatment options were evaluated and included speech therapy, psychotherapy, biofeedback (with limited success), systemic medication (limited success except in abductor cases), nerve section (with late failure rate), and the use of botulinum toxin (improvement in all 34 injected patients).

Adult↗