[Electrodiagnosis of focal neuropathies]. / Elektrodiagnostik fokaler Neuropathien.

Electrodiagnostic techniques used in an EMG-Laboratory and the diagnostic value in focal mononeuropathies are presented. The electrodiagnostic techniques include nerve conduction studies and needle electrode examination. Measurement of distal latency, amplitude, conduction velocity and the finding of focal conduction block may localize the focal character of the mononeuropathy. The needle electrode examination gives further information about distribution, extent, activity, or chronicity of the disease, especially in cases with axonal injuries. Some normal electrodiagnostic patterns are presented, accompanied by some illustrative pathological cases.

Drug induced double peak waveforms in the N20-component of somatosensory evoked potentials.

The double peak waveform of the N20 component of the early somatosensory evoked potentials is a rare finding. In the present paper we investigated 9 patients (mean age +/- SD: 31.3 +/- 11.3 years; range 18-52 years) with the phenomenon of the two subcomponents of the primary cortical complex, after stimulation of the median nerve in a retrospective analysis. The results of the heterogenous patient group showed that the generation of the second subcomponent is not pathognomonic for patients with severe head injury, that it could be reversible and that pharmacological induced effects are presumably responsible for this phenomenon.

Brainstem auditory evoked potentials in respiratory insufficiency following encephalitis.

Brainstem auditory evoked potentials (BAEPs) were recorded in 14 artificially ventilated patients (12 males, 2 females; mean age 33.3 +/- 16.3 years, range 18-67) with respiratory insufficiency resulting from severe inflammatory encephalopathies. The results were compared with those of 17 healthy volunteers (13 males, 4 females; mean age 27.4 +/- 5.3 years, range 21-45). BAEPs in the study patients showed prolonged interpeak latencies (I-III, I-V, III-V, IV-V) and delayed absolute latencies of waves I, II, III, and V at least on one side. Because the auditory pathways are in the near vicinity of the respiratory control centers in the brainstem, the electrophysiologic abnormalities of wave III and the IV/V complex may be a reflection of the disturbed central control of ventilation.

Laboratory diagnosis of radicular and pseudoradicular syndromes in cerebrospinal fluid (CSF): reliability of methods in consideration of pathogenetic aspects. Report on section "CSF diagnosis" of 4th Klagenfurter Neurology Workshop Conference on radicular and pseudoradicular syndromes, Klagenfurt, Austria, August 28-29, 1992.

Laboratory tests may be used to confirm the clinical differentiation of pseudoradicular syndromes and radicular syndromes. In the presence of pseudoradicular syndromes, CSF and blood samples yield no positive results with either non-specific or specific methods. Radicular syndromes give rise to positive findings; using non-specific methods they can be subdivided into inflammatory and non-inflammatory forms, with and without blood-nerve barrier impairment. Non-specific quantities of CSF routine diagnosis are total protein, albumin, leukocyte counts and differential cell count, L-lactate, intrathecal -IgG, -IgA, -IgM and immunoglobulin-class oligoclonal bands. Oligoclonal bands enable the highly sensitive differentiation of non-inflammatory from subacute-chronically inflammatory forms of radicular syndromes. Most of the specific quantities are the subject of current research, e.g. bacterial antigens, D-lactate, cultivation tests, polymerase chain reaction tests and pathogen-specific oligoclonal bands. Pathomechanisms affecting the permeability of the blood-nerve barrier to increasing concentrations of protein and to leukocyte subsets possibly explain the CSF findings in radicular and pseudoradicular syndromes.

[Computer controlled brain death documentation in the intensive care unit]. / Computerkontrollierte Hirntoddokumentation an der Intensivstation.

An interactive, knowledge-based computer system for brain death documentation is presented. The specific exponents BRAINDEX R and G were realised by the software tool Personal Consultant Plus and the programming language Clipper, respectively. The strategies of conclusion were forward chaining for approximate evaluation of coma stages and backward chaining for analysing the brain death syndrome. BRAINDEX was developed for use with an IBM personal computer or compatible equipment. Systemic analyses were compared retrospectively with the data from clinical brain death protocols (n = 132) of 128 comatose patients (mean age 35.1 +/- 15.8 years) with a Glasgow Coma Score of 3. Identical classifications (system vs physician) were found in all patients without diagnosis of brain death (n = 35). Differences related to the findings of the physician were evaluated in lower numbers of the systemic positive diagnosis of brain death (82 vs 89) and higher numbers of impossibility of systemic evaluation (11 vs 2). These results were obtained by conclusions of the computer system drawn by restrictive systemic mechanisms to avoid false-negative diagnoses. The system therefore seems to be useful for documentation, consultation, and as a teaching instrument and data bank in brain death.

Cerebral sinus thrombosis in a patient with hereditary protein S deficiency: case report and review of the literature.

Hereditary protein S deficiency is an established risk factor for venous thrombosis. The common sites of thrombosis are the deep leg and pelvic veins. We report on a 38-year-old female patient with hereditary protein S deficiency and a previous history of deep leg vein thrombosis, who developed thrombosis of the cerebral straight and superior sagittal sinus while taking oral contraceptives. The diagnosis was established by computerized tomography and carotid angiography. Lysis of the thrombus occurred during heparin treatment. The hereditary nature of protein S deficiency was documented by family studies, since nine additional family members deficient in protein S were identified. Nineteen published cases of cerebral vein thrombosis and a deficiency of either anti-thrombin III, protein C, or protein S were reviewed. Compared with patients without a deficiency state, the clinical features of cerebral vein thrombosis were similar except for an earlier onset and a positive medical history of venous thromboembolic events in a considerable number of patients.

Brain death: timing of apnea testing in primary brain stem lesion.

In a 73-year-old patient complete areflexia of the cerebral and peripheral nerves following the rupture of an aneurysm of the basilar artery was diagnosed. During apnea testing the spectral analysis of electroencephalography (EEG) revealed an irreversible shift of peak from 6 to 3 Hz within the low-frequency bands. These findings suggest that apnea testing in patients with primary lesion of the brain stem should be carried out only after an isoelectric EEG.

Cortical evoked potentials by stimulation of the vesicourethral junction: clinical value and neurophysiological considerations.

In 21 healthy volunteers and 42 patients with either neurogenic bladder dysfunction (24), partial peripheral denervation of the bladder (12) or nonneurogenic bladder dysfunction (6) scalp-derived evoked potentials after stimulation of the vesicourethral junction (cortical evoked potentials) were recorded. In addition, evoked potentials from the posterior tibial nerve (tibial somatosensory evoked potentials) and from the pudendal nerve (pudendal somatosensory evoked potentials) were evaluated. The results obtained in normal subjects were reproducible and comparable to those reported in previous studies. Cortical evoked potentials of vesicourethral junction consisted of a prominent negativity with a mean latency of 95 msec. Tibial and pudendal somatosensory evoked potentials were similar and showed a typical W-shaped complex. In normal subjects stimulation of the vesicourethral junction was described as a stimulus-synchronous pulsation combined with a continuous burning feeling and sometimes with a desire to void. In 4 normal subjects no cortical evoked potentials of the vesicourethral junction could be obtained because of a decreased pain threshold. In regard to clinical value, the results demonstrate that in patients with lesions of the central nervous system (in the group with cauda equina and conus medullaris lesions, and in the group with suprasacral spinal cord lesions) the results of cortical evoked potentials of the vesicourethral junction and pudendal somatosensory evoked potentials widely correlate due to similar afferent nervous pathways within the central nervous system. However, in patients with partial peripheral denervation of the bladder with suspected additional secondary local detrusor damage the results of cortical evoked potentials obtained by stimulation of the vesicourethral junction differ mostly from the results of somatosensory evoked potentials obtained by stimulation of the pudendal nerve. The pattern obtained (increased sensory and pain threshold, normal cortical evoked potentials of the vesicourethral junction with normal latencies and normal or increased amplitude) is indicative of local detrusor damage. In 21 patients the ability to detect cortical evoked potentials of the vesicourethral junction was combined with the sensation of stimulus-synchronous pulsation, whereas in the other 21 patients the absence of this sensation during stimulation was combined with the absence of cortical evoked potentials. On the other hand, no correlation was found between the ability of obtaining cortical evoked potentials of the vesicourethral junction and the stimulus-induced sensation of pain and/or desire to void.(ABSTRACT TRUNCATED AT 400 WORDS)

Endocrine abnormalities in severe traumatic brain injury--a cue to prognosis in severe craniocerebral trauma?

Patients with severe craniocerebral trauma (sCCT) display metabolic and endocrine changes. The question is raised whether hormonal patterns give cues to the prognosis of outcome or not. In 21 patients the function of the adrenocortical, gonadal, thyroid and human growth hormone (hGH)-insulin system was assessed. LH, FSH, TSH, prolactin and hGH were stimulated. 3 groups of patients were formed. Group I: patients in acute phase with a Glasgow Coma Score (GCS) more than 6 (group Ia) and less than 6 (group Ib). Group II: patients in transition to traumatic apallic syndrome (TAS). Group III: patients with full-blown or resolving TAS. The values of group Ia comprised low T3, T4 and testosterone, elevated insulin, normal hGH. Group Ib had hypothyroid T3 and T4 and an attenuated response of LH, TSH, prolactin and hGH to stimulation. Group III: there was seen an endocrine normalisation with elevated T4 and TBG and an altered response of hGH and prolactin to stimulation. Endocrine abnormalities were not helpful in predicting which course, either to better or to worse, a given patient would follow.

BRAINDEX: an interactive, knowledge-based system supporting brain death diagnosis.

BRAINDEX (Brain-Death Expert System) is an interactive, knowledge-based expert system offering support to physicians in decision making concerning brain death. The physician is given the possibility of communicating in almost natural language and, therefore, in terms with which he is familiar. This updated version of the system is implemented on an IBM-PC/AT with the expert system shell PC-PLUS and consists of about 430 rules. The determination of brain death is realized with backward chaining and for the optional coma-scaling a forward-chaining mechanism is used.

[Modifications in the Klagenfurt brain death protocol]. / Anderung des Klagenfurter Hirntod-Protokolls.

The Klagenfurter Protocol for determining brain death changed in some criteria during a 4 year period of use. The last version contains non-invasive diagnostic measures and includes the so-called isolated brainstem death.

[Diagnostic criteria in classical infantile subacute necrotizing encephalomyelopathy (Leigh's disease)]. / Diagnostische Kriterien bei der klassischen infantilen subakut nekrotisierenden Enzephalomyelopathie (Leigh's disease).

Subacute necrotizing encephalomyelopathy (SNE; Leigh's disease), though a defined entity in neuropathological and morphological terms, is characterized by high clinical heterogenity. SNE of infancy can be defined and diagnosed on the basis of clinical symptoms more readily than juvenile and adult forms. Four patients with SNE displayed combinations of recurrent vomiting, difficulty in swallowing, failure to thrive, impairment of ocular innervation, muscle tone regulation and central regulation of respiration. These symptoms, particularly in combination, point to a disorder of the brainstem and basal ganglia. In addition, all four patients were suffering from lactic acidosis. Other possible indicators, but not in all 4 patients, were abnormal CT brain scans, impaired nerve conduction velocity, elevated CSF protein levels and enlarged mitochondria in muscle cells. Abnormal brainstem auditory evoked potentials have proved to be one of the best criteria for early diagnosis of brainstem lesions. Enzyme assays of pyruvate degradation in cultured skin fibroblasts revealed diminished activity of the pyruvate dehydrogenase complex in one patient (52 pmol/mg protein x min; median range 313, 82-917, n: 58). This paper summarizes the findings and proposes primary and secondary criteria of assistance in establishing an initial clinical diagnosis of infantile SNE. As no common pathogenetic mechanisms have yet been recognized and no uniform diagnostic markers are yet available, the diagnosis still requires confirmation by histological examination of brain and brainstem, as was the case with all four patients presented.

Short latency somatosensory evoked potentials and brain-stem auditory evoked potentials in coma due to CNS depressant drug poisoning. Preliminary observations.

In patients in coma due to severe CNS depressant drug overdose the central somatosensory conduction time (CCT) after median nerve stimulation is prolonged and N20 is dispersed. Brain-stem auditory evoked potentials demonstrate delayed interpeak latencies (IPLs) I-III, III-V and I-V. This was observed in 4 out of 5 patients investigated after intake of an overdose of amitriptyline (2 cases), barbiturates, meprobamate and nitrazepam (one case each). Toxic levels of drug overdose were related to prolonged CCT and IPLs, whereas normal CCT and IPLs were found at therapeutic drug plasma levels. CCT, IPLs and dispersion of N20 decreased during the course of coma. All patients were successfully treated. It appeared that SSEP and BAEP investigations could make a distinction between a 'toxic' and a 'therapeutic' coma level in severe drug overdose. It further appeared that normalization of CCT and IPLs preceded clinical improvement.

Central somatosensory conduction time and acoustic brainstem transmission time in post-traumatic coma.

Short-latency evoked potentials (SEPs) of the scalp and neck after median nerve stimulation and acoustic brainstem evoked potentials (BAEPs) were recorded in 85 patients in post-traumatic coma with clinical signs of brainstem impairment between days 2 and 6 after trauma. The central somatosensory conduction time (CCT), the amplitude ratio (AR) N20:N13, the interpeak latencies (IPL) I-III, III-V, I-V, and the ARs between waves I and V (I:V) and between wave I and the wave IV/V complex (I:IV/V) were calculated and related to the outcome of the patients. In cases of coma due to supratentorial lesions, CCT and ARs of SEPs were close to normal in patients with good outcome: CCT increased and ARs decreased with worsening of outcome. In cases of primary brainstem injury, a significant prolongation of CCT was also seen in patients with good recovery, whereas normal CCTs could be found in patients with severe disability and death outcome. In this case, unilateral absent scalp SEPs were frequently found. The IPLs I-III, III-V, I-V, and the ARs of BAEPs increased with worsening of outcome. Significant differences of IPL I-V and III-V (brainstem transmission time) were seen between patients with good recovery or moderate disability outcome and the patients with severe disability or death outcome. There was no difference in BAEPs between patients with primary brainstem lesion and patients with secondary brainstem lesion. Patients with bilateral absent SEPs and bilateral absent BAEPs not related to traumatic or preexisting hearing disorders died or survived severely disabled. Unilateral absence of scalp SEPs and unilateral absence of BAEPs were frequently found in patients who died or who had severe disability. Asymmetries in scalp SEPs appeared to be distributed equally to all outcome categories, but asymmetries in BAEPs increased with worsening of outcome too. In most of the patients who died or survived disabled, both SEPs and BAEPs were abnormal.

Carotid occlusion caused by seat belt trauma.

Surgical reconstruction should be considered in patients showing episodes of transient cerebral ischaemia or progressive neurological deterioration secondary to carotid arterial injuries proved by arteriography. Even complete thrombosis of the internal carotid artery may be managed successfully, if thrombectomy and intimal repair are undertaken within some hours after the onset of neurological symptoms. Unger reported a mortality rate of 21% following carotid arterial trauma, 34% of the patients improved if they underwent surgical repair, whereas only 14% of the patients improved if they had ligation or were not treated surgically. Statistical data have shown that both shock and coma are bad prognostic omens and patients presenting with these signs have less than a 50% chance leaving the hospital alive and well, even if they receive optimum emergency management. Although coma has been suggested as a contraindication to carotid repair, review of the literature has shown the data to be too limited to warrant such a conclusion.

[Hypophyseal abscess and cerebral arteritis in a fatal course of pneumococcal meningitis]. / Hypophysenabszess und zerebrale Arteriitis bei tödlich verlaufender Pneumokokkenmeningitis.

Even today uncomplicated courses of pneumococcal meningitis show relatively high fatality rates. Abscess formation leads to a drastic deterioration of prognosis; the same applies to the rarely observed occurrence of a cerebral arteritis. Up to 1985 only 50 cases of pituitary abscess had been described in the literature. This case report describes the signs and symptoms as well as the neuroradiological and post mortem findings on a patient who succumbed to a pneumococcal meningitis, complicated by formation of a pituitary abscess and cerebral arteritis.

[Differential diagnosis of spinal processes using modern diagnostic procedures]. / Differentialdiagnose spinaler Prozesse mittels moderner diagnostischer Verfahren.

Modern diagnostic management of diseases of the spinal cord is discussed, illustrated by some exemplary cases and reviewed by the literature. Special attention is directed to high resolution tomography, magnetic resonance imaging, analysis of the cerebrospinal fluid and electrophysiological methods. It seems conceivable that a shift from invasive methods to noninvasive procedures (e.g. magnetic resonance imaging) may occur.

[How much reintegration can be achieved in patients after severe craniocerebral injury?]. / Inwieweit gelingt eine Reintegration bei Patienten nach einem schweren Schädel-Hirn-Trauma (SHT)?

152 patients, who had suffered head and brain injury during the years 1980 to 1982 were interviewed by letter using a standardized questionnaire to assess the individual outcome. There were questions concerning activities of daily life, invalidity, work activities, social relationships with family and other persons, leisure activity and the grade of persisting sequelae. There were 106 replies, 16 patients had died meanwhile. According to their neurological symptoms on admission at the intensive care unit the patients were divided into two groups. Group I included patient, who showed symptoms of midbrain syndrome stage 1 and 2, while group II presented patients had midbrain syndrome stage 3 and 4. Activities of daily life (feeding, dressing, toiletting and ability to get out of bed and move around the room) were restored in the great majority of patients. 15 patients of group II were classified as invalid. Significant changes in social relationships with the family were seen in 50% of the patients. Further, a decrease of contacts with friends, and a significant decrease in leisure activity (sport) was noted. 28.4% of the patients of group I and 36.8% of the patients of group II, who were at work before the trauma, now were out of work. The others generally worked below their previous level. Persisting sequelae included headache, lack of concentration and weakness of extremities. These symptoms were more prominent in the patients of group II; surprisingly little complaints concerned sexual life and somatic problems. It was found, that severe head and brain injury had significantly changed the patient's life in most cases. Work activities were strongly impaired in about 30% of cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Secondary cerebral amebiasis due to infection with Entamoeba histolytica. A case report with computer tomographic findings.

The clinical and serological findings and computer tomographic (CT) appearances of a case of secondary cerebral amebiasis due to Entamoeba histolytica are presented. The effect of therapy on the neurological symptoms as well as on the CT findings are described in detail. The multiple intracerebral lesions resolved almost completely and the patient survived without any neurological sequelae.