[Prophylaxis of the central nervous system diseases].
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Biomedical subjects
Publications and source records attributed to E Jankowicz.
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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.
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.
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.
The authors describe clinical and electroencephalographic analysis of 23 cases of banign infantile epilepsy. The characteristic features of seizures, the clinical course, and the EEG findings with presence of spikes and/or spikes and slow waves in leads from the motor and midtemporal areas are discussed. Frequent influence of factors releasing the first and subsequent seizures and a history of risk factors during pregnancy, and perinatal period, and early acquired predisposition were demonstrated. Follow-up showed that seizures disappeared at the age of puberty. All observations suggest the possibility of separation of benign infantile epilepsy as a particular form of this disease.
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.
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.
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.
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.
In a material of 14 cases of funicular myelosis the authors demonstrated frequent presence of peripheral signs, psychic changes and rare occurrence of such infrequent syndromes as transverse myelitis, cerebellar syndrome, optic nerve atrophy. Diagnostic difficulties are discussed in cases of pernicious anaemia without blood changes, without gastric achylia, or in patients with vitamin B12 deficiency, malabsorption syndromes and other more infrequent pathological conditions. The importance of such investigations as Schilling's test in atypical cases and the necessity of regular, long-term substitutive treatment with B12 are stressed.
Sixty-five cases of status epilepticus in 51 children were analysed. In 8 cases permanent damage to the CNS resulted and 4 children died. The analysis indicates that the factors influencing the frequency of status epilepticus and CNS damage include: young age of children, infections and their type, withdrawal of antiepileptic medication and the time of beginning treatment of status epilepticus as well as the type of drugs used. In prevention of repeated seizures the authors suggest early administration of anticonvulsants with dehydrating drugs, antipyretics and anti-inflammatory agents. The authors suggest also increasing of the dosage of anticonvulsants in cases of enen common infections beginning in epileptic children.
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 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.
The problem of acquired aphasia during epilepsy in children is discussed with reference to the correlations between aphasia, epileptic seizures and EEG changes as well as diagnostic difficulties and diagnosis.
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.
In 63 patients with brain ischaemia and 29 healthy subjects the rosette test was done and clinical observations were conducted parallelly. The percent of peripheral blood lymphocytes forming rosettes spontaneously was 53.7% in the group of patients, while in the control group it was 63.4%. The difference was statistically significant.
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.