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Biomedical subjects

J Fiksa

Publications and source records attributed to J Fiksa.

5 recordsLinked to original sources

[Paraneoplastic encephalitis].

Paraneoplastic neurodegenerative diseases are defined as damage to central and peripheral nervous tissue related to a malignancy, in the absence of metastases. If they represent first disease manifestation, the diagnosis can pose a real problem. A case of 59-year-old woman is described, who suffered during her last nine months of life from meningoencephalitis, with cranial nerves pareses and progressive quadruparalysis. The CSF exhibited a temporary inflammatory response with slow improvement, whereas the clinical status progressed continually toward terminal bronchopneumonia. An extensive infectious agent search was negative. Though the result of the autoantibody panel available (anti Yo, Hu, Ri) was negative, the paraneoplastic degeneration remained a part of the differential diagnosis. Post-mortem examination revealed residual brain stem meningoencephalitis and advanced cerebellar loss of Purkinje cells, in the presence of a neuroendocrine small cell lung carcinoma in the right lower lobe. The carcinoma only manifested intravitally, with paratracheal lymphadenomegaly on imaging. Rapid progress in the diagnostics of autoimmune neurodegeneration with the increasing spectrum of autoantibody detection tools has recently increased the possibilities of revealing clinically silent, primary manifesting neurodegeneration. In the future, it may also represent a target for therapeutic intervention.

Carcinoma, Small Cell↗

[Cardiac problems in patients with progressive muscular dystrophy].

Progressive muscular dystrophy causes both skeletal and significant cardiological changes. Electrocardiographic and echocardiographic examinations were provided in 30 patients with muscular dystrophy (17 of them with progressive muscular dystrophy Duchenne type, 13 with skeletal muscular dystrophy). In 50% cases were found left ventricle filling disorder, in two cases echocardiographic signs of pulmonary hypertension. ECG showed in one third of cases incomplete right bundle branch block, supraventricular tachycardia was also frequently found.

Adolescent↗

[Ventilatory support in neurologic diseases].

Many neurological diseases cause permanent ventilatory insufficiency. In the ambulatory care we consider regular observation of these patients as a very important. The detection of the early clinical symptoms initialises early ventilatory support. We prefer non-invasive mechanical ventilatory methods to invasive ones. Invasive home care ventilation is very difficult and needs adequate social, medical and economical conditions. We present one case.

Adult↗

Visual and somatosensory evoked potentials in hereditary motor-sensory neuropathies.

Visual (VEP) and somatosensory evoked potentials (SEP) were studied in 31 patients with hereditary motor-sensory neuropathy (HMSN)--in the absolute majority of cases HMSN type I. Prolonged P100 latency was found in the two oldest patients only, though the amplitude of that wave was significantly lower in the adult patients compared with the controls. Typical SEP findings were poorly detectable waves N9,N13 and spinal wave with prominently prolonged N20 und P40 latencies. The amplitude of these cortical components again decreased in proportion to the patients' age. Surprisingly, the greatest involvement of the peripheral sensory and motor nervous systems with the most prominent SEP alteration was found in the youngest generation while in the previous generations there was, in contrast, a more conspicuous component of central affection.

Adolescent↗

[Visual and somatosensory evoked potentials in hereditary motor-sensory neuropathies].

Visual (VEP) and somatosensory evoked potential (SEP) were examined in 31 patients with hereditary motor-sensory neuropathy (HMSN); in the great majority HMSN type I was involved. A prolonged latency of P100 was found only in the two oldest patients, the amplitude of this wave was, however, in adult patients, as compared with the control group, significantly reduced. On examination of SEP a typical finding were N9,N13 waves which were difficult to assess and a spinal wave with markedly prolonged latencies of N20 and P40. The amplitude of these cortical components declined with advancing age. Rather surprisingly the most severe affection of the peripheral sensory and motor neuron was found in the youngest generation of children, while in the older generation of parents the central affection was more marked.

Adolescent↗