Abolished phosphaturic response to parathormone in adult patients with Fahr disease and its restoration after propranolol administration.

The similar localization of intracranial calcification in hypoparathyroidism and in Fahr disease without parathyroid gland disorder suggests that in these two disorders the pathomechanism of calcium phosphate deposition in the brain may be similar. It may be that in Fahr disease some factors, such as chronic respiratory alkalosis, could lead to hypoparathyroidism-like changes in the brain tissue. Abolition of the phosphaturic response to parathormone (PTH) was recently demonstrated in acute experimental hypocapnia. In three adult patients with Fahr disease, a tendency towards compensatory respiratory alkalosis and arterial hypocapnia was found. The parathormone test revealed a marked decrease in phosphaturia response to PTH, but normal cAMP response. In one patient, the parathormone test was repeated during propranolol administration and showed a considerable improvement in the phosphaturic response to parathormone. It is postulated that chronic hyperventilation and hypocapnia as well as phosphaturic resistance to PTH, intracellular increase of phosphate concentration and development of hypoparathyroidism-like intracranial calcification in patients with Fahr disease could all be caused by disturbance of adrenergic receptors and their relationship to PTH receptors.

Primary malignant lymphoma of the brain.

A primary lymphoma in the left temporal lobe of the brain in a 66-year old man is presented. The brain tumor was diagnosed on the basis of clinical symptoms (temporary dizziness, mixed aphasia and right hemiparesis) and repeated CT scanning of the brain. The patient with diagnosed malignant, non-operative brain tumor underwent a series of radiotherapy and steroidtherapy. The patient died twelve months after the first signs and symptoms had occurred. A general autopsy did not reveal any neoplasmatic changes. A macroscopic neuropathological examination showed large tumor's mass in the left temporal lobe which was not separated clearly from its surrounding. A microscopic examination included histopathological (HE, Kanzler-Arendt, Perdrau, PATH, PAS) and immunocytochemical techniques (GFAP, anti-galactocerebroside, LCA, CD45RO, OPD4, CD20). It was found that the examined brain tumor was B-cell malignant lymphoma with the presence of T-cells. Since no changes were revealed in internal organs, the tumor was diagnosed as a primary brain lymphoma.

[History of discoveries and advances in the studies of parkinsonism]. / Historia odkryc i postepu w badaniach nad parkinsonizmem.

A review is presented of the more important discoveries and advances in the 175-year history of the studies and therapeutic trials on parkinsonism. The personality and achievements of James Parkinson are described, with a brief discussion on his most important paper "An Essay on Shaking Palsy". The important works of clinicians, neuropathologists and pharmacologists in the 19th and 20th centuries, especially in the "era of L-DOPA", are reviewed. The development of neurosurgical methods of parkinsonism treatment, including intracerebral grafting of dopamine-producing cells, is outlined.

[The role of enzymatic systems in pathophysiology and pharmacotherapy of Parkinson's disease]. / Rola ukladów enzymatycznych w patofizjologii i farmakoterapii choroby Parkinsona.

In the metabolism of one of the main CNS neurotransmitters dopamine very important roles are played by four enzymes: two synthetizing dopamine: TH and DDC, and two enzymes breaking down dopamine: MAO-B and COMT. In a short review the author discusses disturbances of these enzymatic systems in parkinsonism and therapeutic possibilities of using drugs stimulating or inhibiting the activity of these systems.

[Dopamine agonist in the treatment of Parkinson's disease]. / Agonisci dopaminy w leczeniu choroby Parkinsona.

In the review paper is discussed the group of dopamine agonists which act directly on the postsynaptic receptors in the striatum, and have been used since over 20 years in the treatment of various stages of Parkinson's disease. For practical reasons they are divided in the paper into three groups: drugs used formerly and now gradually withdrawn mainly because of various adverse effects, new drugs whose effectiveness and usefulness have not yet been confirmed clinically, and three drugs (bromocriptine, lisuride, pergolide) used fairly widely with clinically confirmed effectiveness. The mechanism of their action and clinical results are described.

[Epidemiology of epilepsy in school children at Pruszkow and Ursus]. / Epidemiologia padaczki u dzieci w wieku szkolnym w Pruszkowie i Ursusie

The purpose of the study was calculation of the prevalence rate of epilepsy in school-children (7-15 years) in Prusków and Ursus on the basis of cases recorded in specialistic medical sources and determination and description of elementary clinical and social features in the studied group of children. The calculated prevalence rate of epilepsy in the studied population is 6.73/1000. It is higher in boys (7.45) than girls (5.96) and higher in older children - above 10 years (7.52) than in younger children (5.81). Approximate evaluation of the number of epileptic children at school-age on the basis of this rate shows that in the year 1970 there were about 34000 epileptic children registered in medical institutions in Poland. The analysis of all clinical and social data and certain differences in comparison with data in the literature may show that in childhood epilepsy recorded groups of children are formed by the process of selection. Of a significant role in the formation of these groups may be clinical as well as non-clinical factors. It may be expected, thus, that the actual prevalence rate of epilepsy in children population is higher than that calculated from registered cases.

[Clinical assessment of therapeutic results of Ergolactyn (bromocriptine) in Parkinson's disease]. / Ocena kliniczna wyników leczenia preparatem "Ergolaktyna" (Bromokryptyna) chorych z parkinsonizmen.

The effect of Ergolaktyna (bromocriptine) produced by Grodzisk Pharmaceutical Works POLFA on the intensity of parkinsonism manifestations was studied in 27 patients in four clinical centres. All patients had moderately advanced parkinsonism and prior to Ergolaktyna they received L-Dopa preparations which were also given concomitantly with that preparation. The final assessment was done after 4 months of treatment in 18 cases. A good effect of the drug was found in 55% of cases (reduction of the intensity of parkinsonian signs, increased fitness and life activity). Adverse effects developed in 4 cases and required drug withdrawal.

[Tolosa-Hunt syndrome (ophthalmoplegia dolorosa)]. / Zespól Tolosa-Hunta (oftalmoplegia bolesna).

In the reported case unilateral external ophthalmoplegia developed in a young woman, with concomitant pain and anaesthesia in the area innervated by the 1st branch of the trigeminal nerve. After ruling out an expanding lesion of the central nervous system, aneurysm, diabetes, myasthenia, multiple sclerosis and other diseases dexamethasone was administered in a total dose of 30 mg. Complete clinical remission was achieved. In the light of observations and a survey of the peritinent literature it has been accumed that the disease was caused by a non-specific process in the small vessels in the vicinity of the cavernous sinus.

[Progressive supranuclear palsy, diagnostic problems in the light of own case]. / Postepujace porazenie nadjadrowe. Uwagi na temat rozpoznawania--na marginesie opisu wlasnego przypadku.

A case is reported in which the neurological findings and disease course meet fully the criteria of the possible progressive supranuclear palsy. The authors discuss the principles of clinical diagnosis of the syndrome, characteristic oculomotor disturbances and parkinsonian syndrome especially pronounced in this disease in the muscles of the nape, trunk and upper extremities. The main features of neuropathological changes are described fibrillary degeneration and atrophy of neurons in midbrain and pons, with accumulation of tau protein in the neurons and astrocytes in the areas involved by the process.

[EEG pattern during treatment of parkinsonism with L-dopa]. / Obraz EEG podczas leczenia L-dopa chorych z zespolem parkinsonowskim

The authors studied the dynamics of EEG changes in 27 patients treated with L-dopa. The records obtained before treatment were compared with those made during treatment and with the clinical manifestations. In 8 cases the EEG pattern was normal before treatment and 6 of them improved (three-fourths of this subgroup). In 19 cases the EEG pattern was abnormal and 10 of them improved (about 50%). Some intensification of temporal episodic complexes in EEG records was observed during treatment without concomitant changes of clinical manifestations. In 2 cases with slowed down background activity in initial records psychic disturbances of hallucinatory obnubilation type developed requiring withdrawal of treatment. This psychotic reaction was characterized in EEG with increased intensity of generalized slowing down of EEG activity.

[Clinical trial of the first Polish levodopa preparation in the treatment of Parkinsonian patients]. / Kliniczna ocena pierwszego polskiego preparatu lewodopy w leczeniu chorych z parkinsonizmem.

In three neurological centres the therapeutic effects and adverse effects were compared of two levodopa preparations combined with carbidopa (25/250): 1. A foreign drug widely prescribed in Poland, and 2. Its Polish analogue produced by Polfa Stargard Works. The drugs were given to 30 patients (in two matched groups) with Parkinson disease or syndrome during 6 weeks. The trial was completed by 28 patients (one patient dropped out from each group for reasons not directly connected with the treatment). The therapeutic results were assessed on the basis of effects obtained in the NUDS and NSP scales and by measuring of the "on" phase at the beginning and at the end of the trial. In every case the global assessment of patient's condition by the patient and by the doctor was considered. The analysis of the results showed no significant statistically differences between both drugs respect to antiparkinsonian action and incidence of adverse effects.

[Clinical-electrophysiological pattern of juvenile myoclonic epilepsy]. / Ocena obrazu kliniczno-elektrofizjologicznego w mlodzienczej padaczce mioklonicznej.

18 patients (9 females, 9 males) with juvenile myoclonic epilepsy (JME) were studied. Despite a fairly long duration of the disease (from 1 to 34 years--mean 9.5 years) the patients had not been properly diagnosed. In all cases routine serial EEG examinations, 24-hour EEG with Medilog System 9000, and polygraphic Video/EEG/EMG recording were done (Videometry Processor, Glonner). Typical attack triads occurred in 7 cases (39%), grand mal seizures and myoclonic attacks in 10 cases (56%), one patient had only myoclonic seizures. EEG demonstrated already in routine recording occurrence of seizure activity with spike/polyspike-slow wave 3-6 Hz complexes. 24-hour EEG made possible demonstration of these complexes in the remaining 4 cases. Seizure activity during clinical myoclonic seizures were recorded in 8 cases, and during absences in 2 cases. A valuable method was also Video/EEG/EMG recording which showed occurrence of both myoclonic seizures (in 5 cases) and absences (9 cases). Three patients with absences were not aware of their seizures. In 8 patients EEG findings demonstrated focal abnormalities which contributed to previous diagnostic errors. The described clinical and electrophysiological features met fully the diagnostic criteria of JME. In the Polish literature this is the first report on such a large groups of JME cases. Attention is called to diagnostic difficulties due to poor knowledge of this disease and its manifestations which leads to inaccurate history taking. Another cause is non-availability of full neurophysiological diagnostic facilities in many centers.

[Differential diagnosis in idiopathic generalized epilepsies with tonic-clonic seizures: assessment of the use of ambulatory EEG and video/EEG monitoring]. / Diagnostyka róznicowa w padaczkach idiopatycznych uogólnionych z napadami toniczno-klonicznymi--ocena uzytecznosci monitorowanych badan EEG i Video/EEG.

The value of long-term cassette EEG (24-EEG) and Video/EEG for differential diagnosis and classification of idiopathic epilepsies with generalised tonic-clonic seizures (GTCS) was evaluated in twenty-eight patients. The analysis of clinical and EEG features allowed proper classification of epileptic syndrome in twenty-two (79%) patients. In twelve cases absences or myoclonic seizures appeared beside GTCS after 1-9 years from epilepsy onset (mean 3.3 yrs). EEG and clinical data allowed to classify epilepsy in nine (75%) of those patients: in six patients as juvenile absence epilepsy and in three as juvenile myoclonic epilepsy. Sixteen patients suffered from GTCS only (mean duration of epilepsy 10.6 years); in thirteen of them (81%) the diagnosis of epilepsy with GTCS on awakening (AGM) could be established. 24-EEG and Video/EEG helped to demonstrate (a) interictal generalized spike/polyspike-wave discharges (SW/PSW) 3-6 Hz not present in routine EEG in 25% of patients, (b) typical circadian distribution of discharges in AGM patients and (c) absences and myoclonic seizures in 32% of patients. Slow spike-wave variants and focal changes in EEG which could suggest secondarily generalized GTCS were the main diagnostic problem.

[Laser-spectroscopic analysis of pseudocalcium deposits in Fahr's syndrome]. / Analiza laserowa zlogów rzekomowapniowych w zespole Fahra.

Laser spectrography was used for analysis of cations in vascular and tissue deposits in a case of Fahr's syndrome. The following cations were found: calcium, iron, silica, magnesium, aluminium, copper, zinc, phosphorus. In the tissue outside the deposits only magnesium, iron and aluminum were found, and the degree of blackening of spectral lines was much weaker. The authors think that these observations could contribute to a better knowledge of the aetiology and pathogenesis of Fahr's syndrome.

[Treatment of parkinsonism with L-dopa and peripheral decarboxylase inhibitor]. / Leczenine parkinsonizmu L-dopa i inhibitorem dekarboksylazy obwodowej

In 38 patients with Parkinson's syndrome Madopar preparation was used (L-dopa with peripheral decarboxylase inhibitor) in 33 cases as the main drug and in 5 cases as an addition to L-dopa. In the group of 33 patients 39 could complete the treatment, one patient died suddenly, three had the treatment withdrawn in view of side effects. The effectiveness of Madopar was assessed by means of five-rate scoring systems NUDS and ART. Clinical improvement was found in 22 cases (about 67%). The improvement included mainly bradykinesia and rigidity, while tremor was only slightly improved. Side effects developed in about 40% of patients and were slight and transient (apart from 3 cases). The main contraindications seem to be psychotic disturbances. In the group of 5 cases treated with Madopar as an additional drug in low doses improved the result of long-term treatment with L-dopa.

[Effect of Coretal Polfa (Oxprenolol) on parkinsonian tremor and benign essential tremor]. / Wpllyw Coretalu -- Polfa (oksprenolol) na drzenie parkinsonowskie i na drzenie samoistne

Coretal -- an agent blocking the beta-adrenergic receptors -- was given to 12 patients with Parkinson's syndrome with evident tremor and to 4 patients with benign essential tremor. In the group of Parkinson's syndrome the effectiveness of this treatment was evaluated by the blind method. The whole period of observation was 6 weeks, during 3 weeks the patients received Coretal 60-120 mg daily, during the next 3 weeks they were given placebo. The intensity of tremor was assessed by means of a scoring system. Improvement was achieved in 7 patients with Parkinson's syndrome and in 3 out of 4 cases of idiopathic tremor. Complete disappearance of tremor was never observed. Transient side effects were observed in 2 causes. The authors think that Coretal may be used with good result in treatment of parkinsonian tremor and benign essential tremor.

[Epilepsy and other convulsive states in long-term EEG monitoring]. / Padaczka i inne stany napadowe w wielogodzinnym monitorowanym badaniu EEG.

The authors report the results of EEG monitoring lasting many hours with cassette recording and with interpretation after reproduction on monitor screen. The Medilog 9000 System Oxford was used for that purpose. The group comprised 32 verified cases of epilepsy and 19 cases of seizures with unestablished aetiology. In epileptics the method made possible determination of the actual frequency of seizures and their clinico electroencephalographic qualification. In the remaining group of 18 patients the epileptic character of their seizures was demonstrated in 3 cases, in 9 cases the mechanism of these seizures varied, sometimes they were neurotic vasovagal. In 6 cases the monitoring failed to provide sufficient data. These results demonstrate a highly usefulness of cassette EEG monitoring in neurological practice, especially in case of seizures. A valuable supplementation of these investigations are records monitored by television, which make possible establishing of a more close correlation between clinical symptoms and EEG findings.

[Evaluation of the biological availability of 2 carbamazepine preparations]. / Ocena dostepnosci biologicznej dwu preparatów karbamazepiny.

The biological availability was determined of two carbamazepine preparations: Amizepin POLFA and Tegretol GEIGY in tablets. The study was carried out in 10 patients giving them the drug in doses of 400 mg by the crossover method at an interval of 3 weeks. The statistical analysis of the mean maximal values, mean times of reaching of maximal concentrations, and mean areas under the curve of serum carbamazepine concentration changes for both forms of the drug showed a comparable biological availability of Amizepin and Tegretol. The relative degree of bioavailability of Amizepin amounted to 98% of that of Tegretol. The values of pharmacokinetic parameters of both drugs were compared. The value of distribution volume was greater in the case of Tegretol, and the difference was statistically significant. No significant differences were found between the remaining calculated parameters.

[Clinical electroencephalographic diagnosis of myoclonus in various types of epilepsy]. / Diagnostyka kliniczno--elektroencefalograficzna mioklonii w róznych rodzajach padaczki.

Twenty-six epileptics, 16 males and 10 females, with various types of myoclonia were studied. In all patients routine EEG, 24-hour EEG and/or Video-EEG, also with EMG recording were done. Combined detailed clinical-electrophysiological analysis was possible owing to the modern methods of diagnostic examinations and it enabled five epileptic syndromes to be isolated in this group, that is: childhood epilepsy with absence attacks (CAE), juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), progressive myoclonic epilepsy (PME) and photogenic epilepsy. Clinical features of four types of myoclonia occurring in these cases are described in detail since they make possible differential diagnosis of epilepsy syndromes. This is important since the diagnostic and therapeutic methods differ in these cases, and similarly the prognosis varies in these epilepsy types. Stress is laid on juvenile myoclonic epilepsy which is as yet too rarely or erroneously diagnosed.

[Role of the parathyroid glands in the pathogenesis of intracerebral calcifications]. / Udzial przytarczyc w patogenezie zwapnien sródmózgowych.

Investigations of calcium-phosphate metabolism were carried out in a group of 11 children and adults with intracerebral calcifications. It was possible to isolate three different pathogenetic types of calcifications in the striatum and dentate nuclei in the cerebellum. The authors suggest restriction of the term "Fahr's syndrome" to cases without true of false hypoparathyroidism. The assessment of the calcium-phosphate metabolism, and particularly, the test with parathyroid hormone, seem to be an indispensable element in the differential diagnosis of this type of intracerebral calcifications.