[Idiopathic autonomic neuropathy (pandysautonomia)]. / Samoistna neuropatia autonomiczna (pandysautonomia).

On the basis of current literature, clinical and neuropathologic features of idiopathic autonomic neuropathy is presented. Idiopathic autonomic neuropathy is a disease characterized by acute or subacute onset, monophasic course over a period of several years, it is often preceded by an infection. The spectrum of autonomic changes ranges from cholinergic or adrenergic dysfunction to pandysautonomia, leading to heterogeneity of its clinical features. Possible sympathetic system abnormalities found in autonomic neuropathy are: poor pupillary response to light in darkness, orthostatic hypotension leading to syncope, hypotension without compensatory tachycardia, ejaculation disturbances and vasomotor instability. Possible parasympathetic dysfunctions are: salivation and lacrimation disturbances, absent pupillary constriction to light and near gaze, gastrointestinal tract immobility and impairment of gastrointestinal function, atonic bladder with large residual volume, erectile impotence. Pandysautonomia is thought to result from an immune mediated mechanism and responds well to plasmaferesis and intravenous immunoglobin therapy leading to gradual, sometimes not full, recovery. Moreover in this article we pay attention to the clinical value of many tests like cardiovascular or pharmacological studies in the diagnosis of pandysautonomia and in differentiation of pre- and postganglionic changes. In order to diagnose idiopathic autonomic neuropathy one has to rule out a large number of diseases with autonomic dysfunction e.g.: diabetes, malignant neoplasms, acute intermittent porphyria, Shy-Drager syndrome, Riley-Day's dysautonomia, Parkinson's disease, amyloidosis and others.

[Frontotemporal dementias]. / Demencje czolowo-skroniowe.

Frontotemporal dementias are the second largest degenerative dementia group after Alzheimer's disease. It is a clinical syndrome corresponding to at least three histological entities: Pick's disease, non-specific frontotemporal degeneration, frontal lobe abnormalities associated with motor neuron disease. There are four group of symptoms in the clinical description of FTD: behavioural disorder, affective symptoms, speech disorders, neurological signs. FTD is associated with primary degeneration of the frontal and temporal lobes. Histologically there was neuronal loss, microvacuolation, tau- and ubiquitin-immunoreactive inclusions. The ballooned cortical neurons and tau- and ubiquitin-immunoreactive, argyrophilic inclusions have been called Pick-type histology. There are many descriptions of association of FTD and Pick's disease with motor neuron disease and amyotrophic lateral sclerosis. Histological changes were similar to cortical ones. In this study, we described clinical characteristic features of frontotemporal dementia and difficulties in its identification. The distinctive histopathological pattern in the FTD patients and its value to differentiate frontotemporal degeneration from other degenerative dementias is discussed.

[Cortico-basal degeneration]. / Zwyrodnienie korowo-podstawne.

Cortico-basal degeneration (CBD) or cortico-basal ganglionic degeneration is a condition characterised by selective cortical atrophy of parietal and in a lesser extent, frontal lobe associated with dysfunction of the basal ganglia. The clinical symptoms of CBD, predominantly extrapyramidal signs (bradykinesia and rigidity) and apraxia, affect often only one body side in the onset phase, with the left one being more frequent. Neuropathological studies reveal neuronal loss, gliosis, and achromasia chiefly in frontal and parietal cortex, as well as in basal ganglia and substantia nigra. Functional investigations, such as SPECT, disclose similar distribution of abnormalities (hypometabolism). The aetiology and causative treatment of CBD are unknown. The authors highlight the diagnostic difficulties in CBD including a necessity of a prolonged patient's observation in order to ascertain the differential diagnosis of other neurodegenerative disorders, in particular progressive supranuclear palsy, Alzheimer's disease and Parkinson's disease.

[Sensory neuronopathy]. / Neuronopatie czuciowe.

The authors present the question of sensory neuronopathy which are disorders affecting intervertebral ganglia. The neuropathological background and clinical symptoms of sensory neuronopathy are emphasised, as well as diagnostic difficulties resulting from a variety of ethiological conditions: toxic, inflammatory, and autoimmunological ones, and from lack of unequivocal clinical criteria enabling a difference diagnosis along with neuropathy and radiculopathy, which in turn requires a broad spectrum of diagnostic tests and prolonged observation of patients. The authors discuss also the clinical outcome, prognosis, and current therapeutic possibilities focusing on intensive immunosuppressive management.

[Subcortical infarcts (caudate nucleus) in a case of bilateral anterior cerebral artery occlusion]. / Zawaly podkorowe (jadra ogoniastego) w przypadku obustronnej niedroznosci tetnic mózgu przednich.

The authors describe a patient with bilateral anterior cerebral artery (ACA) occlusion. CT and MRI revealed bilateral encephalomalacia in the regions supplied by Heubner arteries and/or by perforating branches of ACA. The patient presented mainly with frontal symptomatology resulting from caudate nuclei lesion. Frontal symptomatology due to caudate impairment is discussed in the sense of frontal-subcortical circuits: lateral orbitofrontal and anterior cingulate ones. We emphasise a similarity of behavioural and cognitive disorders in early Huntington's disease and in frontal lobe lesion.

[Paroxysmal dyskinesis and epilepsy]. / Dyskinezy napadowe a padaczka.

Based on the available literature, we outline the classification criteria of paroxysmal involuntary movements with instant onset and termination. Four types of those movements are currently distinguished: dystonic Mount-Reback paroxysmal choreoathetosis, kinesigenic paroxysmal choreoathetosis, exercise-induced paroxysmal dystonia, and hypnogenic paroxysmal dystonia. We present the clinical characteristics of those entities, as well as diagnostic and therapeutic aspects. An association with epilepsy is emphasised, which seems to predominantly apply to hypnogenic dystonia and kinesigenic paroxysmal choreoathetosis.

[Spontaneous dissection of cerebral arteries. I. Carotid arteries and their branches]. / Samoistne rozwarstwienia tetnic domózgowych. I. Tetnice szyjne i ich rozgalezienia.

Artery dissection means tearing apart of its layers by blood coming inside after endothelial damage. The authors describe the prevalence of that pathology of carotid, arteries (diagnosed rarely so far), pointing to early onset age and to its multifactorial aetiology and pathogenesis. They also outline the pathomechanism of the neurological symptoms and emphasize the variety of the clinical manifestations. The diagnostic possibilities of artery dissection are also presented.

[Spontaneous dissection of carotid and vertebral arteries. II. Vertebral arteries and their branches]. / Samoistne rozwarstwienia tetnic domózgowych. II. Tetnice kregowe i ich rozgalezienia.

Dissection of the extra- and ntracranial portions of vertebral arteries, as well as basilar artery and their branches are discussed based on the up-to-date literature. Formerly, arterial dissections were found exclusively at necropsy, yet they have currently arisen to the field of interest of not only neurologists but also radiologists, neurosurgeons, and vascular surgeons. Ischemic strokes frequently result from arterial dissection, in particular subintimal dissection (most often in the extracranial portion of vertebral artery), by formation of emboli or reduced perfusion. Conversely, subadventitial and transmedial dissections produce aneurysms (most often in the intracranial portion of vertebral arteries and basilar artery) which may result in subarachnoid or intracerebral hemorrhages. The other possible mechanism of focal lesions is the compression of adjacent structures by dissected artery, most frequently by dolichoectasia of basilar artery which originates from its multiple intramural lesions. The dissections may present with cranial nerve palsies, symptoms of ischemia of cervical spine, cerebellum, and cerebral trunk, as well as occipital lobe syndromes. Arterial dissection may occur in adolescents and young adults. The clinical symptom that should be stressed is headache which precedes the occurrence of stroke for several days. The right diagnosis gives a chance for proper treatment, including operative procedures, and improved prognosis.

[Subcortical dementia and the frontal lobe syndrome]. / Demencja podkorowa a zespól czolowy.

Subcortical dementia is a clinical syndrome incorporating disorders of cognitive and affective sphere, which is caused by organic damage to subcortical structures. The syndrome is usually connected with Progressive Supranuclear Palsy, Huntington Disease, Parkinson's Disease. Subcortical dementia is mainly characterized by: slowing down of psychic functions and impairment of their precision, disorders in the ability to use achieved knowledge and personality changes. Most authors stress the fact that similar cognitive and emotional personality defects are observed in cases of frontal cortex damage. Recent research points to the existence of functional subcortical-prefrontal circuits which regulate human behaviour. There is a link between subcortical dementia and functional or structural break of one or more cortical-subcortical connections. Attention is also called to disorders in certain neurotransmitting systems (dopaminergic, acetylcholinergic) as well as to brain hypometabolism in basal ganglia, thalamus and prefrontal cortex.

[Chronic inflammatory demyelinating polyradiculoneuropathy]. / Przewlekla zapalna demielinizacyjna poliradikuloneuropatia.

The authors presented the case of a 32-year-old male patient with a 22-year history of chronic inflammatory demyelinating polyradiculoneuropathy. They paid attention to the number of recurrences and the effects of corticosteroid therapy. They also emphasized that only the peripheral nervous system was affected.

[Cough headache and vertigo as symptoms of the Arnold-Chiari syndrome]. / Kaszlowy ból glowy i zawroty glowy jako wyraz zespolu Arnolda i Chiariego.

Headache with paroxysmal vertigo were induced by cough in a case of the Arnold-Chiari type I malformation. The authors suggest that exertional headache and posterior cranial fossa symptoms of unclear aetiology may indicate presence of the syndrome, and the availability of magnetic resonance imaging made possible the detection of the malformation.

[A case of progressive bulbar paralysis in a child (Fazio-Londe disease)]. / Przypadek postepujacego porazenia opuszkowego u dziecka (choroba Fazio-Londe).

The reported case was diagnosed in a girl aged 14, in whom the first signs developed at the age of 6, with progressive involvement of the cranial nerves IX, X, XI and XII, and V with VII. After ruling out other possible causes the diagnosis of the Fazio-Londe disease was established. Electromyographic examination confirming the diagnosis demonstrated also evidence of changes of neurogenic type in the muscles of upper extremities despite absence of demonstrable clinical abnormalities, which, as suggest the authors, may support the hypothesis of the similarity of the disease to other forms of spinal muscular atrophy, such as Werdnig-Hoffman and Kugelberg-Welander diseases.

[A case of epidermal nevus (Jadassohn's phacomatosis) with changes in the nervous system]. / Przypadek znamienia naskórkowego (fakomatozy Jadassohna) ze zmianami w ukladzie nerwowym.

A 22-year old patient with Jadassohn's naevus phacomatosis affecting the right side of the head, face and brain is reported. Besides naevus linearis on the forehead, lipomata of the right palpebra, skin and palate, 2 odontomata, hypodermal and submucosal hyperplasia of the right half of the oral cavity, a small aneurysm of the internal carotid artery in the cavernous sinus and linear calcification in the cortex of the medial surface of the occipital lobe like those in Sturge-Weber disease were found. Clinically, she was found to be mentally retarded (moderately) and having epilepsy. Epileptic attacks occurred up to the age of 13 years, while changes in EEG are still present.

[A case of crossed aphasia in a right-handed person]. / Przypadek afazji skrzyzowanej praworecznych.

A 17-year-old boy, right-handed, and without a family history of left-handedness is described. Occlusion of the right internal carotid artery caused in him left-sided hemiparesis with aphasia. The cross-aphasia of right-handed individuals is discussed in the light of literature reports.

[Detailed monitoring of valproic acid and phenobarbital in the treatment of epilepsy in children]. / Szczególowe monitorowanie kwasu walproinowego i fenobarbitalu w leczeniu padaczki u dzieci.

24-hour monitoring of the treatment of three children with refractory epilepsy with valproic acid and phenobarbital was carried out. This made possible a detailed analysis of the variability of valproate level in the serum over 24 hours and no relation was found between the level of the drug and its dose. A rise of phenobarbital level was observed when it was given with valproic acid, and this was associated with a reduction of seizure activity in the EEG. In view of a high variability of the valproate level over 24 hours detailed monitoring refractory cases seems useful and indispensable sometimes.

[Rheoencephalographic evaluation of the relations between the changes in cerebral circulation in the areas supplied by specific arteries in patients with cerebral stroke]. / Wspólzaleznosc zmian krazenia mózgowego w obszarach poszczególnych tetnic w ocenie reoencefalograficznej chorych z udarami mózgu.

Rheoencephalography was made in 105 patients with ischaemic brain stroke during the first phase after the onset of the disease and 10 days later. It was found that the presence of an ischaemic focus in the area of the internal carotid artery induced blood supply alterations within the areas of adjacent++ arteries, i.e. contralateral carotid artery and vertebral arteries. In some cases it became visible in the reg records under the form of reduced or enhanced pulsation of blood flow followed by weaker reactions of changed vascular tonus. Most distinct changes were observed at the onset of the brain stroke. Ten days later these changes became less expressed.