[Positron emission tomography and evoked potentials in the early detection of hereditary ataxias].
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Biomedical subjects
Publications and source records attributed to W F Haupt.
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We compared two selected groups of patients with acute Guillain-Barré syndrome requiring artificial ventilation. Both groups were treated with conventional therapy, group A (n = 35) between 1970 and 1978, group B (n = 21) between 1979 and 1983. Neither plasmapheresis nor any other immunosuppressive treatment was applied. Significant differences were found favouring group B: There was a decline of mortality from about 60% to less than 10% (p less than 0.001) and a smaller number of severe complications due to improvement of supportive care.
Nineteen patients with various forms of chronic polyneuritis of the Guillain-Barré type were attributed by uniform clinical criteria to the different subgroups and their electroneurographic findings followed during the clinical course. The follow-up studies were conducted for 1 to 12 years with a mean of 5.3 years. All patients were treated with steroids and/or Azathioprin, in addition, six patients were subjected to plasma exchange therapy. Of the 13 patients without plasma exchange four demonstrated improvement of electroneurographic parameters, four were unchanged, two deteriorated. The six patients treated with additional plasma exchange showed improvement in four cases and deterioration in two cases. No correlation was found between clinical findings and long-term electroneurographic data. Improvement of electroneurographic data was usually less impressive than the clinical recovery. Following plasma exchange treatment three patients showed remarkable, however only temporary improvement of electroneurographic data which also did not correlate to the long-term outcome. Therefore, the long-term follow-up of electroneurographic findings renders no decisive prognostic statement in chronic polyneuritis.
The prognostic implications of bilaterally abolished cortical median-nerve evoked somatosensory potentials were examined in a retrospective study of 255 patients with neurological disease requiring intensive unit treatment. In 57 cases, we encountered bilaterally abolished somatosensory potentials. Of these patients, 54 died during the course of hospital treatment, 3 other were referred to other hospitals in the state of irreversible apallic syndromes. We did not encounter the reappearance of once abolished somatosensory cortical potentials. It follows from these findings that the bilaterally abolished somatosensory potentials are correlated with a very poor prognosis. In several selected cases the special aspects of patients with circumscript infratentorial lesions are discussed.
In 38 patients suffering from spontaneous pontine hemorrhages arterial hypertension was the most common risk factor. 31 patients, of whom 30 died, suffered centro-paramedian pontine hemorrhage leading to coma, hypertensive crisis, respiratory failure, cardiac arrhythmia, miosis and tetraparesis. Most cases showed involvement of the mesencephalon [17] and fourth ventricle [14]. The other seven patients demonstrated dorsotegmental [5] or hemipontine [2] hemorrhages with complex neuroophthalmologic signs, other cranial nerve lesions, and ataxia or hemiparesis; all these patients survived, some even without neurological deficit. In most cases, arteriosclerotic hemorrhages of pontine vessels occurred; in rare cases arterial malformations [4] and anticoagulants [4] were responsible for the bleeding. Clinical signs, CT scans and MRT investigations led to the diagnosis. EEG and evoked potentials allowed statements regarding localisation and prognosis. Treatment was limited in most cases to conservative intensive care; in one case a ventricular shunt was implemented, and in two cases pontine hemorrhages in the presence of arteriovenous and cavernous angiomas were removed.
In neurological patients with severe cerebral disease requiring intensive care unit treatment serial examinations of brainstem acoustic evoked responses and median nerve evoked cortical somatosensory potentials regularly allow the detection of typical constellations which can be attributed to primary supra- or infratentorial disease. These findings are of diagnostical use but also allow prognostical statements. Here, somatosensory evoked potentials are of great value. In a series of 255 patients, all 57 showing bilaterally abolished somatosensory potentials did not recover. Therefore, the demonstration of bilaterally absent somatosensory potentials in supratentorial cerebral disease invariably designates poor prognosis.
Two cases are reported: one with an asymmetric polyneuropathy involving the left lower extremity, the other with a generalised chronic relapsing-remitting demyelinating polyneuropathy, hitherto not described in this context. The findings are discussed with regard to the relevant literature.
Between 1972 and 1984, 20 cases of the supinator syndrome were examined with clinical and electrophysiological methods in our clinic. In 12 cases we found an idiopathic supinator syndrome in the strict sense and in 8 cases a traumatic lesion of the r. profundus of the radial nerve. We carried out follow-up studies in most of the patients. From these findings we attempt to derive guidelines for treatment. An absolute indication for operative revision in our opinion is present only in the case of complete transsection by trauma. Otherwise, the spontaneous course can be observed. The operative results in idiopathic cases are poor; it seems to be purposeless to operate patients who have a paresis for a long time. Therefore, the decision for or against an operative intervention has to be made soon after the diagnosis is secured.
The criteria of brain death published by the Federal Chamber of Physicians in 1982 in their second generation have stood the test of time and remain valid. The most important amendments in issuing a third generation are the following: In primary cerebral lesions supratentorial lesions have to be distinguished from infratentorial processes. In primarily the infratentorial processes EEG activities of the cortex max outlast apneic brain stem areflexia. Therefore in infratentorial processes controls of the EEG are indispensable. The extinction of evoked potentials, certain conditions being observed, may prove the loss of brain stem function and therefore may allow to shorten observation time, and may offer an alternative to isoelectricity in the EEG or cerebral perfusion stop as a diagnostic procedure. Additional tests for brain death are commented on. Special considerations are necessary in brain death in children.
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A 64-year-old Cambodian male with lepromatous leprosy of several months' duration, with only brief previous treatment, had symmetrical patchy loss of pain and thermal sensibility including the lateral foot regions. Quantitative assessment by light and electron microscopy revealed normal counts of myelinated fibers (MF) and an increased number of unmyelinated fibers (UF). The respective abnormal histograms and the qualitative differentiation of UF-associated Schwann cells suggested a combination of segmental demyelination with Wallerian degeneration of MF, and a loss of genuine UF, concomitant with considerable regenerative activity of both fiber populations.
According to the recommendations of the medical board of the FRG the clinical diagnosis of cerebral death in adults requires an observation period of 12 hours. Evoked cerebral potentials have been accepted as a technical method of confirmation of clinical signs. In 32 patients with the signs of apneic brain-stem areflexia acoustic evoked brain-stem potentials and median-evoked somatosensory potentials were recorded. In supratentorial lesions evoked potentials are adequate to confirm complete cessation of brain function. In three cases of primary brain-stem disease evoked potentials were abolished, however, unequivocal EEG activity was preserved. The registration of evoked potentials is sufficient to confirm the brain death syndrome in primary supratentorial lesions, in infratentorial disease, however, demonstration of isoelectric EEG is necessary.
Following a review of pertinent literature, nine cases of chronic polyneuritis are reported, who underwent a total of 21 series of plasma exchange treatment during immunosuppressive medication. The effect of treatment was variable. Four out of 7 patients with relapsing and/or stepwise progressive disease demonstrated reproducible improvement of neurological signs. The three others responded to a lesser degree. Two patients with a chronic progressive course experienced only mild improvement. In general, the reaction to plasma exchange did not correlate with duration and severity of the previous clinical course, reaction to immunosuppressive drugs, or electrophysiological and morphological findings in muscle and nerve biopsies. Moreover, a clear correlation with pathogenic effects of patient-IgG in animal experiments ("passive transfer") was not discernible.
The origin of spontaneous myogenic activity during the registration of isoelectric EEGs for documentation of total brain death has not yet been explained. In nine patients with total brain death, completely abolished acoustic evoked brainstem potentials (AEP), somatosensory evoked potentials (SEP) as well as electrically evoked blink-reflexes (BR) were demonstrated. The peripheral conduction capacity of the facial nerve, however, was never impaired. These findings suggest that peripheral nerve function may be well preserved although brain stem activity is completely abolished and therefore support the thesis that the myogenic activity in cranial muscles in total brain death is not mediated by the brain stem but is of extracerebral origin.
Polyneuropathy in Tangier disease can be divided into three clinical types. The most severe form (type III) with a syringomyelia-like syndrome has been described in three cases only. Here, a fourth case of this type is presented. Because of unusual trophic disturbances even leprosy was suspected. Electrodiagnostic findings, including evoked cerebral potentials in this case, were suggestive of a generalized neuropathy with some degree of primary or secondary demyelination and implied possible impairment of central structures. Sural nerve biopsy, including electron microscopy and quantitative analysis, revealed a predominant reduction of smaller myelinated and unmyelinated fibres. The main morphological feature was the abundance of abnormal non-membrane-bound vacuoles in Schwann cells, mostly of the unmyelinated type, and in some endoneurial fibroblasts, macrophages and perineurial cells. There was no inverse relationship between lipid vacuoles and axons in Schwann cell complexes as suspected by others. An excess of endoneurial collagen as well as an increased fascicular area were obvious. In five skin biopsy specimens of different regions typical vacuoles were noted in Schwann cells, histiocytes, nevus cells, and rarely in perineurial cells.
The cerebral function of 55 patients with stenosis and occlusion of carotid arteries was monitored intraoperatively by serial examination of somatosensory cortical evoked potentials (SEP). In 40 uncomplicated operations no significant alteration of the SEP was detected. In 13 examination series, however, reversible abnormalities were noted. Furthermore, in two cases irreversible SEP loss was documented. The nature and temporal development of SEP alterations enabled differentiation of possible etiological factors. Bilateral abnormalities were indicative of global hypoxemia whereas acutely arising unilateral SEP loss suggested embolic occlusion of cerebral arteries. Intraoperative SEP monitoring therefore appears to be of diagnostical and differential diagnostical value and allows statements in respect to further intra- and postoperative treatment.
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In a collective of 22 patients (18 adults, 4 children) with the clinical diagnosis of peroneal muscular atrophy the correlation of various neurophysiological parameters was examined. These included electromyography, motor and sensory nerve conduction velocity, visual (VEP), acoustic (AEP) and somatosensory (SEP) evoked potentials and conventional EEG. In all cases we found a high correlation between the findings of nerve conduction velocities and somatosensory evoked responses. Acoustic evoked potentials were examined in 18 cases and showed 10 pathological findings; visual evoked responses were also examined in 18 cases and produced 5 pathological findings. If nerve conduction velocity measurements are possible, SEP examinations are superfluous as they do not provide additional information. On the other hand, VEP and AEP testing is warrented in all cases to document the impairment of central nervous pathways.