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Biomedical subjects

K Jellinger

Publications and source records attributed to K Jellinger.

At least 235 records · Page 13Linked to original sources

Progressive supranuclear palsy: clinico-pathological and biochemical studies.

Ten autopsy cases of Progressive Supranuclear Palsy (PSP) are reported. Age at onset ranged from 16 to 67 years and the duration of illness 3 to 24 years. The clinical features were aggressive mental retardation in 4 cases with early onset, paroxysmal dysequilibrium, ophthalmoplegia, rigidity and akinesia, pseudobulbar palsy and variable degrees of dementia. Neuropathology showed widespread neurofibrillary degeneration associated with system-bound neuronal loss and gliosis in subcortical areas, particularly affecting the subthalamic nucleus, substantia nigra, brainstem tegmentum and dentate nuclei, with no or little involvement of the cerebral cortex. The distribution of the lesions and the ultrastructure of the neurofibrillary tangles made of 15 nm straight filaments (seen in one case) in PSP are different from postencephalitic parkinsonism, Guam Parkinson-dementia complex and brainstem affection in (pre)senile dementia. Post-mortem biochemical analysis of two brains disclosed severe reduction of tyrosine hydroxylase, the key synthetic enzyme of the catecholamine pathway, not only in the nigrostriatal system as seen in Parkinson's disease, but in most areas of the brain-stem and limbic system. The implication and possible pathogenic and therapeutic significance of these biochemical findings are discussed. The etiology of PSP and its nosological position within the degenerative extrapyramidal disorders remain unknown.

Adolescent↗

Symptomatology of the most severe form of tuberculous meningitis.

Seven cases of the most severe form of tuberculous meningitis, in which a midbrain syndrome developed, are reported. Three different types of progress were observed. Exudative inflammation and cerebral edema dominated in the first group, causing the rapid development of the acute midbrain syndrome, which may turn into a bulbar syndrome. In the second group the development of the midbrain was delayed and an apallic syndrome followed. The morphological examination disclosed local diencephalic and midbrain lesions caused by herniation and specific vasculitis and vascular compression. The third group showed disintegration of cortical function as a result of parenchymal lesions, apart from local midbrain symptoms which never fully intensified into the midbrain syndrome. Observation of the progress of the disease proved that late diagnosis and delayed therapy were decisive in cases of the most severe form of tuberculous meningitis.

Adolescent↗

Oral and parenteral nutrition with L-Valine: mode of action.

The intravenous application of the branched chain amino acid L-valine to patients with hepatic coma resulted in a specific drop of tryptophan, serotonin and 5-hydroxyindole acetic acid. Clinical improvement of hepatic encephalopathy and of the underlying neurotransmitter (serotonin) and ammonia derangements by administration of L-valine suggest a possible role of this competitive amino acid on intermediary metabolism and ammonia detoxification.

Aged↗

[Strategies of psychopharmacotherapy in the elderly (author's transl)].

The administration of psychopharmacologic drugs plays an important role in the therapeutic strategies of geriatric age groups. Like most other drugs psychopharmacological substances due to morphological and biochemical aging processes of the brain, the multimorbidity of the elderly, and the age-related differences in pharmacokinetics and pharmacodynamics, they show altered drug responsiveness which has to be considered in the selection and dosage of the psychopharmacological drugs administered. Neuroleptics, antidepressants and tranquilizers are increasingly used in the treatment of symptomatic and endogenous psychoses, reactive psychiatric disorders and sleep disorders in geriatric age groups. Some substances of choice in the treatment of special psychiatric disorders in elderly are discussed. The administration and dosage of psychopharmacolic drugs preclude the knowledge of unwanted side effects of these drugs which show increased incidence in the geriatric patients.

Aged↗

[Pathogenesis and morphology of circulatory disorders of the spinal cord. Review].

After a short recapitulation of the functional anatomy and recent findings with respect to the pathophysiology of the spinal circulation, the more important forms of lesions, sites of disturbances and patterns of the extension of spinal blood flow disturbances are described. The morphological findings and patterns of lesions only give conditioned indications with regard to the site of disturbances causing impaired blood supply and of the clinical symptoms, but it is possible to differentiate certain standard lesions and topical lesion patterns which are demonstrated for processes in the aortic region, affections of root and other aortomedullary affluxes, the spinal longitudinal arteries and the intra- and erimedullary vessels and the venous path and documented by instructive examples. Special emphasis is laid on the role of spinal blood flow disturbances in spinal cord injuries, diseases of the vertebral column as well as in diagnostic and vascular-surgical interventions. Finally, deformations of the spinal vessels and their complications are briefly discussed. The knowledge of the functional anatomy, pathophysiology and pathomorphology of spinal blood flow disturbances appears to be essential for an early diagnosis and a systematic therapy of these rare diseases of the spinal cord which are difficult to demarcate differential-diagnostically.

Animals↗

[Bromazepam in the treatment of somatized psychogenic disorders (author's transl)].

The clinical effects are reported of the benzodiazepine derivative, bromazepam (Lexotanil) in the treatment of psychosomatic disorders in the course of neurotic, psychovegetative, and masked depressive syndromes. The drug was administered orally in 301 patients (178 males, and 123 females). Target symptoms were anxiety, tension, and varied organic dysfunction of psychogenic origin. The optimum daily dosage was three times 3 mg; the duration of treatment ranged from 1 week to 34 months. The effect of treatment was considered excellent in 51.5%, good in 42.5%, moderate in 2%, and absent in 4%. The most responsive target symptoms were psychogenic disorders of the cardiovascular system and of the gastrointestinal tract, as well as anxiety, while no true antidepressive effect was observed. Drug tolerance is excellent. Slight fatigue, vertigo or a mild reduction in psychomotor activity were complained of by about 10% of the patients and usually occurred with daily doses of 18 mg or more, whereas no other side effects were observed. There was no obvious tendency to drug dependence even after after long-term treatment of up to 34 months. Bromazepam appears to be a superior compound to other anxiolytic and psychovegetatively active minor tranquillisers on account of its mild hypnotic action. Its anxiolytic effect causes additional indirect sleep induction in the above-mentioned conditions.

Adult↗

Intramedullary spinal cord metastases.

Seven autopsy cases of intramedullary metastases, four in the cervical spinal cord, are reported and the literature reviewed. Whereas lung and breast cancer, malignant melanomas and lymphomas are reported as the most common primary tumors, the present series included three cases of breast carcinoma and two cases each of colon and oat cell carcinoma of the lung. Neither the clinical symptoms nor the neurological signs distinguished intramedullary metastases from the more common extradural deposits, but radiological evidence of vertebral metastases and myelographic stop were present in only one case each, and CSF cytology was negative. Intramedullary deposits in this series were neither associated with extradural tumor nor with spread into the subarachnoid space, while cerebral metastases were present in four cases. This favors hematogenous dissemination rather than direct transdural or perineural spread of these lesions.

Adenocarcinoma↗

Primary malignant lymphoma of the CNS and polyneuropathy in a patient with necrotizing vasculitis treated with immunosuppression.

A woman, aged 58, who had undergone prolonged treatment with corticosteroids for generalized necrotizing vasculitis, and had received azathioprine (Imurel) for 18 months, 4 years after discontinuing the latter treatment, developed peripheral neuropathy and a rapidly progressing cerebral disorder suggesting a basal meningeal process. CSF cytology suggested malignant lymphoma with meningeal involvement. Immunological studies showed an increase of Null-lymphocytes in the peripheral blood. Autopsy disclosed a primary malignant lymphoma of the CNS with the histological appearance of a multilocular immunoblastoma showing almost ubiquitous meningocerebral involvement. Clinical and postmortem examinations failed to demonstrate any systemic extraneural lymphoproliferative disorder. In addition, there was peripheral polyneuropathy of the axonal type with denervation atrophy of skeletal muscle, but without lymphomatous involvement of the neuromuscular system. The possible relations between primary malignant lymphomas of the CNS and previous immunosuppressive treatment of immunoinflammatory disease are discussed in view of the concept of impaired immunoregulation. The pathogenetic background of peripheral polyneuropathy is unknown.

Azathioprine↗

The symptomatology with the most severe clinical course of spontaneous subarachnoid hemorrhage.

The symptomatology of 18 patients with the severest clinical course after subarachnoid hemorrhage (SAH) is described and analyzed. Seventeen patients died, five with an acute bulbar syndrome with cardiac arrest, and 12 with irreversible breakdown of brain function. One patient had an apallic syndrome with minimal signs of remission, who died 4 months after the first rebleeding. The cases are divided into five clinical groups on the basis of the acute brainstem symptomatology which set in instantly or after temporary adaptation, or following a second hemorrhage. Pathological examination revealed that 16 patients had a brainstem pressure cone, 14 with marked edema and signs of herniation; one patient had only cisternal tamponade while another had predominantly brain edema with herniation. There was striking parenchymatous damage of the brain of the patient with the apallic syndrome. There was a marked analogy between the material reported and the symptomatology described by Plum and Posner (1972).

Adult↗

[Familial striatal degeneration (author's transl)].

An extrapyramidal disorder occurring in three generations of a family (only males) is described The clinical features were progressive dementia and extrapyramidal signs without choreiform hyperkinesia. The youngest patient (onset of disease at the age of 22 years) showed tremor, rigidity, ataxia, convulsions, and myoclonus. The neuropathologic findings were characterized by isolated symmetrical degeneration of the corpus striatum and diffuse cortical atrophy without affecting other cerebrospinal neuronal systems. The clinical features of this familial disorder and its relation to other types of familial striatal degeneration and to the juvenile form of Huntington's chorea are discussed.

Adult↗

Combination chemotherapy (COMP protocol) and radiotherapy of anaplastic supratentorial gliomas.

Postoperative survival time and recurrence-free intervals in 116 consecutive patients with supratentorial grade III and IV gliomas (glioblastomas, gliosarcomas, anaplastic astrocytomas, and ependymomas) were compared in unselected groups receiving different forms of treatment. Postoperative high-voltage radiotherapy (31 patients, dosage 4,000--6,000 rads) and combined chemotherapy consisting of CCNU, vincristine, amethopterine, and procarbazine in 15-day circles (COMP protocol) (12 patients) showed the same median survival time of 10.6 months and comparable recurrence-free intervals of 6.8 and 7.0 months, respectively. These results were significantly different from a control group (39 patients) receiving best postoperative supportive (conventional) care (median survival 5.4 months, free interval 3.7 months). Combination of postoperative radiotherapy with simultaneous polychemotherapy (COMP protocol), evaluated in 18 patients, did not significantly change the recurrence-free interval (median 7.0 months), but increased the median survival time to 12.9 months, which was significantly superior to the two other treatment groups. The toxic side effects of COMP therapy were moderate and essentially haematological. In general, simultaneous radiation and chemical treatment was well tolerated after major tumour resection. These preliminary results of postoperative combination of radiation and polychemotherapy for anaplastic supratentorial gliomas appear encouraging, but further trials for optimization of combined therapeutic strategies are warranted.

Adult↗

Primary brain tumour presenting as spontaneous intracerebral haemorrhage.

In an autopsy series of 430 spontaneous intracerebral haematomas 44 cases, or 10.2 percent, were caused by a proved neoplasm, including 21 anaplastic gliomas, 17 metastases, 2 oligodendrogliomas, 2 malignant lymphomas, and one meningioma. These instances of massive bleeding into brain tumour represented 2.4 percent of about 1,800 primary and secondary cerebral neoplasms proved by necropsy. In only four of the patients with primary brain tumours (two glioblastomas, one oligodendroglioma invading the leptomeninges, and one primary malignant lymphoma), three of them with a history of arterial hypertension, were the presenting symptoms these of a spontaneous intracerebral haemorrhage, and the tumour itself was not diagnosed until surgery or necropsy. One patient with acute haemorrhage into a glioblastoma of the basal ganglia showed a rapidly lethal course, while the others demonstrated one or more episodes before the onset of the acute fatal illness and a prolonged period from the time of the bleed until death. The clinical features and the pathogenesis of spontaneous haemorrhage into cerebral neoplasms are briefly reviewed.

Aged↗