Idiopathic hypoparathyroidism with intracranial calcifications and dominant skin manifestations.

Presented here is an unusual case of a 41-year-old man with idiopathic hypoparathyroidism strongly connected with dermatological, ophthalmological and neurological disorders. Since the age of 4 he had been treated ineffectively for mycosis resulting in complete baldness and atrophic nail plate changes. At the age of 35 he was diagnosed with idiopathic hypoparathyroidism. He underwent surgery twice due to bilateral cataract. CT scans of the head demonstrated numerous symmetrically located calcifications in both frontal lobes, subcortical nuclei, the paraventricular region, brain fornix, and both cerebellar hemispheres. The neuropsychological examination demonstrated the occurrence of psychosensory disorders under the form of hallucinations accompanied by a sense of fear and anxiety. It seems essential to investigate the parathyroid gland for any sign of pathology, especially amongst patients with severe dermatological and/or neuropsychological symptoms. Early diagnosis and treatment of patients with hypoparathyroidism may prevent the development of many serious complications or at least result in marked improvement of neurological manifestations.

Erythromelalgia--a thrombotic complication in chronic myeloproliferative disorders.

Erythromelalgia is a very specific, thrombotic syndrome related with thrombocythemia that may occur during the course of chronic myeloproliferative disorders (MPD), especially polycythemia vera (PV) and essential thrombocythemia (ET). This poorly understood clinical syndrome is characterized by red, congested distal extremities and painful burning sensations, usually confined to the ball of the foot and one or more toes or fingers. If left untreated, it may progress towards acrocyanosis and even peripheral gangrene. Sometimes, it may precede the diagnosis of MPD by months or years. The pathophysiological aspects of erythromelalgia as well as its differentiation with erythermalgia have been reviewed in this study.

Myeloproliferative disorders--neurological complications.

Primary myeloproliferative disorders (MPD) are often associated with hemostasis abnormalities, which may cause many thrombotic or hemorrhagic complications during the course of the disease. Clinical consequences following abnormal hemostatic conditions include various neurological manifestations. It is extremely difficult to predict and evaluate the risk and chance, that MPD patients will develop neurological symptoms. The up-to-date background of pathological thrombocytosis, as well as the neurological aspects of abnormal hemostasis during the course of myeloproliferative disorders have been reviewed in this study.

[Convulsive status epilepticus--clinical analysis of patients treated iin the Neurological Clinics, Medical Academy in Lublin in the years 1986-1995]. / Stan padaczkowy napadów drgawkowych--analiza kliniczna chorych leczonych w Klinice Neurologii AM w Lublinie w latach 1986-1995.

The authors reviewed clinical records of 57 consecutive adults (age: 17-78, 63%--men) treated in the intensive care unit to convulsive SE that was refractory to first-line medication (BDZ,PB). They were divided into three groups: up to 30 (mean 21 years, 28%), between 31-50 (43 y, 32%) and above 50 (59 y, 40%), 58% had previously had epilepsy with prevalence in the youngest (85%). Among the oldest in whom epilepsy occurred de novo as much as 42% experienced it in the form of convulsive SE. Generalized SE was observed in 83% of cases; exclusively in patients up to 50 and in 61% of the oldest. The identifiable precipitating causes of SE were determined in 72% cases but in 25% there were two or more of them. Among previously epileptics leading etiologies for SE were: alcohol abuse, infection or drug withdrawal. Recent brain injury (stroke, neuro-infection, trauma) accelerated refractory seizures in epilepsy-free cases. Time to recovery varied from 0.5-2 (6%) to 2-6 or above 6 hrs (46% each) after continuous i.v. administration of BZD or chlormethiazole (53%) when ineffective. No side effects were noted. The commonest complications during SE were hyperthermia and transient dysregulation of circulatory or/and respiratory systems. Everyone was led out of SE. Overall mortality amounted to 12%. Among the deceased 71% were in the oldest group and everyone with recent brain lesion. This study highlights differences in the course of convulsive SE according to age and underlying etiology and the importance of intense care in therapeutic schedule. A more common chlormethiazole administration, a useful therapeutic tool in management of convulsive SE in adults had been discussed.

[Case of cerebral aneurysm, arteriovenous fistula and cerebral hematoma]. / Przypadek tetniaka, naczyniaka tetniczo-zylnego i krwiaka sródmózgowego.

A female patient is reported who had subarachnoid haemorrhage and in whom angiography demonstrated presence of an aneurysm and an arteriovenous angioma in the territory of the left middle cerebral artery. During surgical intervention an intracerebral haematoma was found additionally in the posterior part of the left temporal lobe. The postoperative course was uneventful. The patient was discharged without evidence of focal brain injury.

[Case of arteriovenous hemangioma, aneurysm and subdural hematoma]. / Przypadek naczyniaka tetniczo-zylnego, tetniaka i krwiaków podtwardówkowych.

In a woman aged 53 years angiography of the internal carotid arteries demonstrated presence of an aneurysm in the region of arteriovenous fistula, which is a very rare occurrence, and coexistence of bilateral spontaneous subdural heamatomas. This coincidence has not been reported.

[Levels of ammonia in patients with stroke]. / Zawartosc amoniaku w krwi zylnej chorych z udarem mózgu.

The authors determined peripheral-blood ammonia level in patients with cerebral strokes. The determinations were done by the method of Konitzer et al. on the 1st, 3rd and 7th days of the disease. The results of determinations were subjected to statistical analysis with the t test of Student. It was found that the mean serum ammonia level in patients with cerebral stroke on the 1st day of the disease was higher than in the control group. The difference was statistically significant. The level of ammonia in the blood of patients with cerebral haemorrhage was significantly higher on the 1st day of the disease than in patients with encephalomalacia. The difference was statistically significant with error probability of 0.05.