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[Clinical picture and roentgenoradiological diagnosis of suprasellar cerebrospinal fluid cysts in children].

Suprasellar cysts containing cerebrospinal fluid are considered a rare pathological condition. Characteristic symptom complexes in children are discussed on the basis of 12 cases. It is assumed that signs of affection of the chiasma and optic nerves in slowly progressive hydrocephalus, diencephalic disorders, and hyperkinesis suggest the presence of a suprasellar cyst. The authors present data on the use of diagnostic methods of examination and give their opinion on the informative value of these methods: craniography, gamma-topography and radionuclide cisterno- and ventriculography, computerized tomography, and computerized-tomographic cisterno- and ventriculography.

Adolescent↗

[Contribution to the neuropathology of stereotactic brain-operations and their fatal complications (author's transl)].

Report on the neuropathology of 6 fatal cases in 150 stereotactic operations, performed on 135 patients from 1960 to February 1978. The deceased had a hyperkinesis and a severe Parkinson's disease in 3 cases each. Only two of them had died of a complicating intracranial extracerebral haemorrhage immediately after the stereotactic operation. In our own material the longest survival time after operation was about 3 years. Biochemical and biothermical reactions and situations basing on thermocoagulation are dealt with in particular.

Adult↗

Patient care and family counseling. Social work considerations in audits of hyperkinetic reaction of childhood.

To assure optimal patient care, a multidisciplinary audit of hyperkinetic reaction of childhood should involve the social work department. Specific process criteria can be established to assess the psychosocial evaluation of the patient, inpatient social work treatment, and follow-up care. Collectively, these criteria provide a mechanism with which to measure psychosocial factors that contribute to hyperkinesis, improvement in the patient's condition, family responsiveness to patient needs, and the effectiveness of the social work department of carrying out its responsibility for treatment of hyperkinetic children.

Child↗

A preliminary study of the effect of pyridoxine administration in a subgroup of hyperkinetic children: a double-blind crossover comparison with methylphenidate.

A small sample of six patients with the putative "hyperkinetic syndrome" participated in a research protocol comparing administration of pyridoxine, methylphenidate, and placeboes. The children had had low whole blood serotonin levels and a history of previous responsiveness to methylphenidate. The results of the double-blind clinical evaluation showed trends suggesting that both pyridoxine and methylphenidate were more effective than placebo in suppressing the symptoms of hyperkinesis. Pyridoxine elevated whole-blood serotonin levels, methylphenidate did not. Clinical and laboratory evidence indicated that the pyridoxine effects persisted after the 3-week period when the vitamin had been given in this experimental design.

Adolescent↗

Nutrition, brain function and behavior.

Current food intake has been shown to directly affect neurotransmission, with resultant modification of behavior. The role of vitamin co-factors in brain function is discussed, with emphasis on changes in mood and neurological function with deficiency. The use of megadoses of vitamins for the treatment of psychiatric diseases has little scientific support at this time. Current research also does not substantiate the Feingold thesis of improvement in childhood hyperkinesis when an additive-free diet is consumed. The effects of medications used to moderate mood are related to changes in nutrient intake that in turn alters weight status. In addition, the effect of certain nutrients modifying the dose response to mood altering drugs has been discussed. Finally evidence for mood state directly affecting the capacity of the body to utilize nutrients is presented.

Adolescent↗

Controversial practices in allergy.

The clinical practice of modern allergy has a sound foundation in a scientific understanding of the pathogenesis of hypersensitivity disorders. However, several practices continue to be regarded as controversial. Controlled studies have failed to establish the effectiveness of intracutaneous or low-level modified radioallergosorbent titration as a guide to immunotherapy. In addition, controlled studies do not support the clinical usefulness of provocative subcutaneous or sublingual testing procedures of the leukocytotoxicity assay for the diagnosis of food allergy. Food additives have not been clearly shown to influence hyperkinesis, and autogenous urine immunotherapy is without supporting evidence of effectiveness and is potentially harmful. Since allergic diseases are common and the economic impact of medical care for these patients is great, it is important for physicians to understand the basis for these procedures and to advise patients accordingly.

Desensitization, Immunologic↗

Effect of phenoxybenzamine, propranolol, phenylephrine and isoproterenol on the circulation of rats bearing Guérin carcinoma.

Effects of phenoxybenzamine (1 mg per 100 g i.v.), propranolol (0.2 mg per 100 g i.v.), phenylephrine (0.1--0.3--1.0--3.0 micrograms per 100 g per min) and isoproterenol (0.01--0.03--0.1--0.3 micrograms per 100 g per min) were studied on cardiac output (Evans-blue dilution), organ and tumour blood flows (Sapirstein's isotope indicator dilution technique) in rats bearing Guérin carcinoma. In agreement with our earlier results data for haematocrit, blood pressure, TPR and organ vascular resistance were lower whereas cardiac index and organ blood flow were higher in control untreated tumour-bearing animals than in untreated normal rats. Phenoxybenzamine, depending on tumour size, decreased blood pressure, TPR and vascular resistance of organs, while it was found ineffective in rats with tumours of very large size (60--80 g). Propranolol treatment produced slight and similar effects in both normal and tumour-bearing rats. Phenylephrine enhanced peripheral resistance as a function of log dose in both normal and tumour bearing animals, nevertheless in the latter group it elicited more marked responses in the vascular bed of the kidneys, intestines and skin. The vasodilatory log dose dependent effect of isoproterenol was not present in tumour-bearing rats. In addition to the anaemia, the continuous decrease of alpha adrenergic activity in the systemic circulation parallel with tumour growth appears to be the most reasonable explanation for the "hyperkinesis" observed in tumour-bearing rats. Resistance of tumour vascular bed was increased by both phenoxybenzamine and propranolol. During the course of phenylephrine infusion (1.0 or 3.0 micrograms per min) the resistance of tumour vessels was more increased than that of other organs. Isoproterenol infusion resulted in a vasoconstriction of the tumour vessel. It appears that with maximally dilated vessels, vasoconstriction remains the only possible vascular response in Guérin carcinoma. In the vascular bed of the tumour a preponderance of beta adrenergic receptors also seems to be feasible.

Animals↗

[The surgical treatment of muscle tonus disorders by the destruction of the input areas of the posterior spinal nerve roots].

Stereotactic cryodestruction of the subcortical ganglions for microdestruction of the inlets of the radices spinales was conducted in resistant to drug and surgical treatment patients with impaired muscular tension and spastic pain syndrome (infantile cerebral paralysis, dystonia musculorum deformans, postencephalitic spastic-hyperkinetic syndrome). A total of 8 patients aged 19-46 were treated. The destruction of the segments depended on the site of the tension impairment. In cervical, upper extremity, lumbar, lower extremity involvement the segments to be destroyed were, respectively: SV--SVII, SVIII, LII--LIII, LIV, LV and SI. The surgery aimed at breaking the centripetal nociceptive and myotactic tracts. The treatment reduced muscular hypertonicity and hyperkinesis, relieved spastic pain. The paper presents anatomic and neurophysiological grounds for the procedure application.

Adult↗

[Neurosis-like tics in children].

The author examined 115 children from 4-14 years who suffered from ticose hyperkinesis. The specific traits of the background (somatical diseases, organic brain diseases, mental disorders, the MBD syndrome permitted to eliminate 4 groups of patients. On the basis of the achieved results the author comes to the conclusion that in the pathogenesis of a pseudoneurotic tic an important role is being played by a premorbid insufficiency of the motor analyzer. Comprehensive treatment is being recommended, with a consideration of the background against which the hyperkinetical syndrome arises.

Adolescent↗

[Tremor as a symptom of organic brain damage in individuals of young age].

Cases of young patients with different diseases of nervous system in which tremor was the main symptom, clinical peculiarities of trembling hyperkinesis in essential tremor, in olivopontocerebellar degeneration, in strionigral atrophia, in residual phenomena of organic brain damages as well as their differences from functional hyperkinesia are characterized. The data are presented on comparative efficiency of different methods of examination.

Adolescent↗

Coloboma contiguous gene deletion encompassing Snap alters hippocampal plasticity.

Mice heterozygous for the semidominant mutation coloboma (Cm/+) display several distinct pathologies including head bobbing, ophthalmic deformation, and locomotor hyperactivity. The Cm/+ mutation comprises a contiguous gene defect which encompasses deletion of the gene Snap encoding the presynaptic nerve terminal protein SNAP-25 that is an integral component of the synaptic vesicle docking and fusion complex. Indeed, SNAP-25 is required for axonal growth and for the regulated release of neurotransmitters at the synaptic cleft. As an extension of our studies on the behavioral deficits exhibited by these mutants, including evaluation of the hyperkinesis and dopamine-related behavioral pharmacology that might be related to attention-deficit hyperactivity disorder in humans, we have studied spontaneous electroencephalographic and evoked potential recordings in the dentate gyrus of halothane-anesthetized Cm/+ and normal (+/+) littermates to evaluate potential physiological abnormalities of synaptic function in these mice. While sensory activation elicited by brief (10 sec) tail-pinch produced 1-2 min of theta rhythmic activity in +/+ mice, theta induction was markedly reduced in Cm/+ mice. There were no significant differences in dentate afferent-evoked population excitatory postsynaptic potential (pEPSP) slopes, pEPSP facilitation, or population spike (PS) amplitudes; however, paired-pulse inhibition of dentate PS amplitudes was significantly increased in Cm/+ mice. Furthermore, although brief high-frequency stimulation of the perforant path produced robust long-term potentiation (LTP) of synaptic responses in the dentate gyrus of +/+ mice, LTP was attenuated in Cm /+ mice. It has been previously demonstrated that dopamine (DA) neurotransmission is essential for induction of one type of hippocampal theta rhythm and also may modulate hippocampal LTP, suggesting that alterations in DA synaptic transmission may underlie the behavioral abnormalities, in particular the hyperactivity, associated with Cm/+ mutant mice.

Animals↗

Diagnosis of Angelman syndrome in infants.

The diagnosis of Angelman syndrome (AS) has seldom been made in infants because the previously described characteristic manifestations usually are not apparent until after age 2 years. We describe 4 AS patients, one of whom has oculocutaneous albinism, who were less than 2 years old when first evaluated. All 4 have deletions of the region q11.2-q13 of chromosome 15. In the 3 cases in which parents were available for study the deleted chromosome 15 was maternally derived, as determined by cytological markers. All of the patients presented with severe to profound global developmental delay and postnatal-onset microcephaly; they had seizures, hypotonia, hyperreflexia, and hyperkinesis. All were hypopigmented as compared to their relatives. Each had eye abnormalities; all had choroidal pigment hypoplasia. None were initially described as having an abnormal appearance. We believe that AS is far more common than previously thought and present these 4 children to emphasize the manifestations that may be helpful in making the diagnosis in the young patient. We also emphasize the hypopigmentation that patients with AS frequently have, including what we think is the first reported case of albinism and AS.

Chromosome Deletion↗

Acute hemodynamic deterioration during rapid atrial pacing in patients with hypertrophic cardiomyopathy.

BACKGROUND AND HYPOTHESIS: Supraventricular tachycardia and ventricular tachycardia are often observed in patients with hypertrophic cardiomyopathy (HCM) and they often alter the clinical features of HCM. We examine the influence of supraventricular tachycardia on cardiac function and assess the clinical characteristics of patients with HCM. METHODS: We studied 32 patients with HCM and 8 normal volunteers using echocardiography under transesophageal rapid atrial pacing. RESULTS: Presyncope-associated hypotension was observed during rapid atrial pacing in 8 HCM patients, but in none of the normal controls. During rapid atrial pacing (144 +/- 8 beats/min in HCM, 146 +/- 5 beats/min in controls), systolic blood pressure (SBP), the product of left ventricular filling volume (FV) and heart rate, and fractional shortening (%FS) in the HCM patients decreased significantly compared with the basal values (138 +/- 19 mmHg vs. 99 +/- 24 mmHg, 5.0 +/- 1.2 l/min vs. 2.9 +/- 0.9 l/min, 41.7 +/- 6.2% vs. 35.2 +/- 6.0%, respectively), but these decreases were not observed in normal controls. The decrement of SBP during rapid atrial pacing in HCM patients with a history of syncope was more marked than that in those without such history. The decrement correlated positively with the indices of left ventricular hypertrophy (maximal wall thickness and wall thickness index) and with %FS, and correlated negatively with the endsystolic left ventricular diameter at rest. CONCLUSIONS: In some patients with HCM, supraventricular tachycardia causes marked hemodynamic deterioration that may be related to a history of syncope, marked hypertrophy, hyperkinesis, small cavity size, and small filling volume of the left ventricle.

Adult↗

The monoamine reuptake blocker brasofensine reverses akinesia without dyskinesia in MPTP-treated and levodopa-primed common marmosets.

The common marmoset develops motor deficits after MPTP treatment and exhibits dyskinesia after chronic levodopa (L-dopa) dosing and subsequent re-challenge with L-dopa and other dopaminergic agents. We report on the actions of the potent monoamine reuptake blocker brasofensine on motor disability, locomotor activity, and dyskinesia in the 1-methyl-4-1, 2,3,6-tetrahydropyridine (MPTP) -treated marmoset model of Parkinson's disease. Oral administration of brasofensine (0.25, 0.5, 1.0, or 2.5 mg/kg) to MPTP-treated marmosets produced a long-lasting, dose-dependent increase in locomotor activity and reduction in disability scores. In addition, coadministration of the lowest dose of brasofensine (0.25 mg/kg orally) with a threshold oral dose of L-dopa (2.5 mg/kg) caused a marked increase in locomotor activity, greater than that produced by either drug alone. In other MPTP-treated marmosets previously primed to exhibit dyskinesia by repeated L-dopa dosing, brasofensine effectively reversed akinesia with a naturalistic and prolonged motor response without the appearance of dyskinesia or stereotypy. This finding contrasts with the severe dyskinesia, stereotypy, and hyperkinesis produced by equivalent doses of L-dopa. The ability of brasofensine to produce a prolonged and naturalistic antiparkinsonian response without eliciting dyskinesia after previous L-dopa priming may relate to actions on D(1) receptor-linked pathways. These findings suggest that monoamine reuptake blockade may be of value in the treatment of Parkinson's disease, both early in the disease course and when L-dopa-induced dyskinesias complicate treatment.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗

Effects of chronic in vivo replacement of choline with a false cholinergic precursor.

Chronic administration to rats of a diet in which all choline is replaced by NADe, an unnatural choline analog, results in a classical hypocholinergic syndrome characterized by progressive loss of learning and memory, hyperkinesis, hyperreactivity and hyperalgesia. Discontinuation of the artificial diet results in rapid elimination of NADe from both free and phospholipid-bound pools in all tissues studied, but the behavioral effects recede more slowly and incompletely. These results are consistent with a model in which choline and NADe compete in both acetylcholine and phospholipid synthesis, resulting in selective vulnerability of cholinergic neurons. Histological studies are in progress to determine whether microanatomical changes are also consistent with this model.

Animals↗

Involvement of brain monoamines in the stimulant and paradoxical inhibitory effects of methylphenidate.

The significance of central noradrenergic, dopaminergic and serotonergic neural systems for the locomotor stimulant effects of methylphenidate was investigated in the rat. In order to study the role of brain catecholamines, rats were pretreated with reserpine (2.5 mg/kg) followed 24 hrs later by treatment with alpha-methyltyrosine (25 mg/kg) or U-14,624 (75 mg/kg), a dopamine-beta-hydroxylase inhibitor. In these experiments, methylphenidate stimulated motor activity was antagonized by alpha-methyltyrosine and enhanced after treatment with U-14,624, suggesting that release of newly synthesized dopamine is important to a locomotor stimulant action of methylphenidate. Evidence implicating brain serotonin in the actions of methylphenidate was obtained in rats pretreated with pargyline or p-chlorophenylalanine (PCPA). Administration of pargyline 1 hr prior to methylphenidate was found to reduce the locomotor activity induced by methylphenidate and this was antagonized by pretreatment with low doses of PCPA. Higher doses of PCPA caused a significant elevation of methylphenidate induced activity which could be reduced by 5-hydroxytryptophan. Destruction of serotonergic neurons with 5,7-dihydroxytryptamine also potentiated methylphenidate induced locomotion. These latter findings suggest that serotonergic fibers have an inhibitory function in brain. These results are discussed in relation to the possible mechanism by which methylphenidate may act in hyperkinesis.

5-Hydroxytryptophan↗

Differential effects of methylphenidate on reticular formation and thalamic neuronal activity.

Intravenous administration of methylphenidate (1 or 2 mg/kg) markedly attenuated the unit discharge rate in the mesencephalic reticular formation of rats and cats. Concurrently this drug enhanced the neural activity in the primary sensory nuclei of the thalamus. The differential effect of methylphenidate on these two neural systems suggest a possible mechanism by which it may improve attentive processes in hyperkinesis.

Acoustic Stimulation↗

Movement disorders induced in monkeys by chronic haloperidol treatment.

After several months of treatment, Cebus apella, Cebus albifrons, and Saimiri sciurea monkeys maintained on haloperidol, in doses of 0.5 or 1.0 mg/kg orally 5 days per week, began to display severe movement disorders, typically 1-6 h post-drug. Cebus monkeys exhibited violent, uncontrolled movements that flung the animals about the cage. Such episodes usually lasted only a few minutes, recurring several times during the period following drug ingestion. Writhing and bizarre postures dominated the response in S. sciurea. Cessation of drug treatment produced no distinctive after-effects. When tested as long as 508 days after the last administration, however, Cebus monkeys responded to haloperidol with several episodes of hyperkinesis, even at challenge doses considerably lower than those in the original treatment.

Animals↗