Acute interventional recanalisation of vertebrobasilar stenoses by angioplasty: complications and 12 months follow up.

INTRODUCTION: In acute symptomatic vertebrobasilar artery stenosis, the use of mechanical recanalisation remains controversial. The complication rate of acute interventional recanalisation (aIR) has to be considered, as evidence from randomised trials is lacking. In a single centre retrospective case series, we here describe complications and outcome after aIR. METHODS: We retrospectively assessed aIR in a tertiary care centre and included the following parameters: indication for aIR, national institute of health stroke scale (NIHSS) score on admission, recanalisation by thrombolysis in myocardial infarction score (TIMI) grades, post-interventional complications, mortality, NIHSS and modified Rankin scale at follow-up and rate of restenosis. RESULTS: We identified 14 aIR (14 percutaneous transluminal angioplasty with or without stent implantation in 12 patients; 6/12 with thrombolysis; n = 6 vertebral artery, n = 8 basilar artery; 4 women, mean age 67 years). Mortality was 25 % (3/12) after 7 days and 42 % (5/12) after 12 months. In 12/14, interventions are complete (TIMI 3, 86 %), in 2/14, a partial recanalisation (TIMI 2, 14 %) was achieved. In one case, a peri-interventional fatal intracerebral haemorrhage occurred (1/12, 8 %). At late follow-up (mean 342 days), one re-occlusion (1/7, 14 %) and one recurrent stroke (1/12, 8 %) were observed. CONCLUSIONS: In our single centre series of vertebrobasilar aIR recanalisation rate was high. However, procedural safety and clinical outcome varied considerably. The results of aIR need to be assessed in multicentric registers to define the procedural risk and outcome in the clinical setting.

[ALS and frontotemporal dementia - case report and review of the literature]. / ALS und frontotemporale Demenz - Fallbericht und Literaturübersicht.

The occurrence of cognitive decline in amyotrophic lateral sclerosis (ALS), especially in the form of frontotemporal dementia (FTD), has been described previously. Recent molecular biology and histopathology data suggest that both ALS and FTD may share common pathological pathways and may present two phenotypes of the same proteinopathy. The underlying pathophysiological mechanism may be defective RNA- and DNA-modulation, mediated by the proteins TDP43 and FUS. These findings are suggestive of a new disease category of TDP43-proteinopathies, which include ALS, FTD and overlap syndromes. While about half of the FTD cases are associated with TDP43-deposits, tau is found in the other half. A significant clinical overlap to other tauopathies exists here as well, for instance with corticobasal degeneration. In this paper, we present a case report and review the clinical spectrum and current pathogenetic concepts of FTD.

Systemic thrombolysis with rt-PA in patients under 40 years of age: a subgroup analysis of the Cologne Stroke Experience.

BACKGROUND: While the application of intravenous systemic thrombolysis (IVT) with rt-PA (recombinant tissue plasminogen activator) in older patients is currently moving into the focus of epidemiological studies, only few data are available regarding the application in young patients ≤40 years. Single-center data of a thrombolysis register were analyzed with respect to safety and efficacy of the treatment of young patients. METHODS: In a retrospective subgroup analysis of 450 patients treated by IVT within a 3-hour time window, patients ≤40 years were identified (n = 20). Clinical data [age, pretherapeutic stroke severity (National Institute of Health Stroke Scale, NIHSS), OTT (onset to-treatment time), rt-PA-dose, DNT (door[-]to[-]needle time), rate of symptomatic intracranial hemorrhages] and medical history were determined. The clinical outcome was assessed by the mRS (modified Rankin Scale). The results were compared to those of patients >40 years (n = 430). RESULTS: Twenty patients ≤40 years (mean age 32 years) out of 450 patients (4%) were treated by IVT. The percentage of predisposing diseases and vascular risk factors was significantly lower when compared to patients >40 years (p < 0.05). In contrast, the percentage of smokers was significantly higher (55 vs. 24%; p < 0.05). In comparison to patients >40 years, OTT, DNT and NIHSS at admission were not significantly different. After 3 months, 11 of 20 young patients (55%) showed a favorable outcome (mRS 0-1) and 80% were functionally independent (mRS 0-2). In the group of patients >40 years (n = 430), the respective percentages were significantly lower [p < 0.05; 34% (mRS 0-1) and 52% (mRS 0-2), respectively]. Symptomatic intracranial hemorrhages were not observed (in patients >40 years: 4%, p < 0.05). CONCLUSIONS: In comparison to the cohort of patients >40 years, IVT in young patients is safe and leads to a significantly better outcome after 3 months. Our data therefore encourage the use of IVT in young patients.

A giant bilateral calcified chronic subdural hematoma.

BACKGROUND: A calcified chronic subdural hematoma is a rare disease and its neuroradiological presentation is variable. The degree of calcification extends from thin calcified inner membranes to dense calcification and even ossification of the hematoma. Previous reports described a maximum of two hematoma cavities with calcified inner hematoma membranes. METHODS: Neuroimaging report with illustrative computerized tomography images. RESULTS: A patient with a bilateral symptomatic calcified chronic subdural hematoma, or so-called "armoured brain", was admitted to our intensive care unit with clinical signs of increased intracranial pressure. Computerized cranial tomography demonstrated multiple bilaterally located hematoma cavities with thin calcified inner membranes. After neurosurgical intervention by bilateral burr hole trepanation, clinical symptoms improved. CONCLUSIONS: Our case of a calcified chronic subdural hematoma presents with an uncommon imaging pattern with more than four hematoma cavities bounded by predominantly convex- and concave-configured thin calcified inner membranes.

Selective immune adsorption treatment of severe Guillain Barré syndrome in the intensive care unit.

BACKGROUND: The effectiveness of plasma exchange and intravenous application of immunoglobulins (IVIG) for the treatment of the Guillain Barré syndrome (GBS) has been demonstrated in large collectives. In contrast, there are only a few investigations in GBS patients with severe symptoms admitted to the intensive care unit (ICU) and treated with selective immune adsorption (SIA). We compared the efficacy and safety of SIA only versus SIA followed by IVIG in patients with severe GBS. METHODS: Patients with severe GBS admitted to the ICU were treated with SIA only or in combination with IVIG. Severity of symptoms was assessed using Hughes grades and severe GBS was defined as ≥ 3. Data were acquired retrospectively for the last 10 years (1998-2008). RESULTS: Data from 30 GBS patients (age 53 ± 16 years) with severe symptoms (Hughes grade 5: 30% [n = 9], grade 4: 57% [n = 17], grade 3: 13% [n = 4]) were analyzed. The mean Hughes grade at admission was 4.2 ± 0.7. Ten patients were treated by SIA only, 20 patients were treated sequentially with SIA followed by IVIG (30 g/d) over 3 days. The number of SIA sessions was 3.2 ± 0.8. Improvement of Hughes grade 4.2 ± 0.7 to 3.4 ± 0.9 (P < 0.001) occurred within 14.6 ± 15.5 days. Treatment with SIA only was as effective as the sequential therapy with IVIG. The Hughes grade decreased significantly in the group of patients where SIA was performed only (P = 0.008) and in the sequential treatment group (P < 0.001), respectively. In one patient SIA had to be terminated after one session due to ICU complications. Other severe side effects were not observed. CONCLUSIONS: In severely affected GBS patients admitted to ICU treatment with SIA seems to be safe and effective. In comparison to treatment with SIA only, sequential therapy with IVIG was not more effective.

Evoked potential monitoring in carotid surgery: a review of 994 cases.

Intraoperative monitoring of brain function is desirable in carotid artery surgery to detect possible complications, but the monitoring methods must be simple to perform, sensitive, and reliable. Median nerve somatosensory evoked potential (SEP) monitoring fulfills these criteria. Between 1985 and 1990, we performed 994 operations of the carotid artery with SEP monitoring. In 92% of the cases, we were able to obtain viable SEP tracings. In seven cases, irreversible SEP loss was followed by a new neurologic deficit. In one case only, neurologic complications ensued without SEP loss. Although immediate intraoperative therapeutic options are limited, the monitoring enhances patient security by allowing intraoperative detection and postoperative analysis of complications. SEP monitoring appears to be at least as effective as conventional EEG monitoring. The viability, sensitivity, and reliability of newer methods, such as modified spectral EEG analysis, must be measured by this established procedure.

Prognosis of acute polyneuritis requiring artificial ventilation.

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.

European brain death codes: a comparison of national guidelines.

The majority of European countries have published recommendations for the diagnosis of brain death as a necessary prerequisite for organ donation. The concept of brain death as defining the death of the individual is accepted in all European countries; however, the guidelines for determining the total and irreversible loss of all brain functions differ somewhat in the various countries. While the clinical examination and documentation of the clinical signs of brain death are very uniform, there are significant differences in the guidelines for using technical confirmatory tests to corroborate the clinical signs. These range from rejecting all technical tests to accepting of multiple neurophysiological tests alone or in combination. The present study examined the similarities and differences in current concepts of brain death in the various European countries and their recommendations for the diagnosis of brain death. The diagnosis of brain death is based on a number of prerequisites, on the clinical diagnosis of deep coma, loss of all brainstem reflexes, and the demonstration of apnea. Neurophysiological tests are recommended by a number of national professional societies as confirmatory tests to confirm the clinical diagnosis of brain death and shorten otherwise necessary waiting periods of 6-12 h. Most brain death codes allow the use of electroencephalography, which must demonstrate electrocortical silence over a certain period. Evoked cerebral potentials can demonstrate the successive loss of activity of various afferent pathways and are accepted in some countries as a confirmatory test. Other neurophysiological tests which demonstrate the loss of cerebral perfusion can be implemented. Brain scintigraphy can confirm the loss of isotope uptake into the brain. Doppler sonography also demonstrates cessation of brain perfusion. Cerebral panangiography may also be used to demonstrate the loss of brain perfusion but is less desirable since it might endanger the patient.

Severe polyneuropathy in Tangier disease mimicking syringomyelia or leprosy. Clinical, biochemical, electrophysiological, and morphological evaluation, including electron microscopy of nerve, muscle, and skin biopsies.

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.

Sequential treatment of Guillain-Barré syndrome with extracorporeal elimination and intravenous immunoglobulin.

Plasma exchange (PE) and administration of intravenous immunoglobulin (IgG) are established treatments for Guillain-Barré syndrome (GBS). Elimination of postulated pathogenetic factors by plasma exchange or similar methods, such as selective adsorption (SA) treatment using affinity-type adsorption columns and subsequent immunomodulation by intravenous IgG, may provide a more effective treatment. Forty-five patients with acute GBS were prospectively examined using a clinical score. We treated 11 patients by plasma exchange, 13 with selective adsorption using a tryptophan-linked polyvinyl alcohol gel adsorbent, and a group of 21 patients by selective adsorption followed by intravenous IgG. The patients treated sequentially by selective adsorption and intravenous IgG improved significantly better than the patients who received plasma treatment only. This pilot study suggests that sequential treatment of GBS may be superior to plasma treatment alone. The higher cost of this combined treatment might be offset by shorter hospital stays and lower overall expenditure. The preliminary results warrant further investigation in a multicenter trial.

Contribution of initial median-nerve somatosensory evoked potentials and brainstem auditory evoked potentials to prediction of clinical outcome in cerebrovascular critical care patients: a statistical evaluation.

In a prospective study of 200 patients with cerebrovascular disease (48 intracerebral hemorrhages, [ICH]; 64 subarachnoid hemorrhages, [SAH]; 48 supratentorial and 40 infratentorial ischemic strokes), we assessed the individual and combined prognostic value of median-nerve somatosensory evoked potentials (SEP) and brainstem auditory evoked potentials (BAEP) within 72 hours of admission. Clinical outcome was graded in three ranked categories according to a modified Glasgow Outcome Scale. Likewise, the initial SEP and BAEP findings were graded in a three-class score. In all groups, the SEP were significantly correlated with outcome (P < 0.01). Likewise, after partialling out the prognostic effect of SEP, the contingency between BAEP and outcome was statistically significant, except in ICH (P = 0.07). The contingencies of SEP and outcome in ICH and supratentorial infarcts were higher than the corresponding partial contingencies for BAEP, while the latter were higher in infratentorial infarction and SAH. Therefore, in all disease groups except for SAH, the multiple contingency coefficients ranging from 0.67 to 0.75 were statistically significant and greater than either simple or partial contingencies alone. The results of the two evoked potential modalities combined permit statistically significant superior prognostication in most cerebrovascular diseases when compared to those of either of the modalities alone.

Serial evoked potentials and outcome in cerebrovascular critical care patients.

Median-nerve evoked somatosensory evoked potentials (SEPs) and brainstem auditory evoked potentials (BAEPs), examined early in the course of patients suffering from cerebrovascular disease, correlate statistically significantly with outcome. Little is known about the changes of evoked potentials in the course of disease and their correlation to outcome. In a series of 215 patients (75 supratentorial infarctions, 36 infratentorial infarctions, 58 supratentorial hemorrhages, 18 infratentorial hemorrhages, and 28 aneurysmatic subarachnoid hemorrhages) requiring neurologic intensive care treatment, we prospectively examined the correlation between the findings of serial SEPs and BAEPs and outcome at 4 weeks. Evoked potentials were examined after admission, after 1 week, and after 2 weeks. The findings were classified in 4 categories (normal, unilateral or bilateral pathologic findings, unilaterally attenuated, and bilaterally attenuated). Clinical outcome was determined by classification according to the Glasgow Outcome Scale (death, persistent vegetative state, severely incapacitated, mildly incapacitated, and recovery). Statistical evaluation was performed using Fisher's exact test for all variables. In all subgroups, SEPs correlated statistically significantly with outcome at all three examinations. No correlation was found for BAEPs at first examination in infratentorial disease, nor at second examination in subarachnoid hemorrhages. In all other cases, SEPs and BAEPs were correlated statistically significantly with outcome at all three examination timepoints.

Cerebral glucose metabolism in acute and persistent vegetative state.

Regional cerebral glucose metabolism (rCMRglc) was investigated with 18F-2-fluoro-2-deoxy-D-glucose (FDG) and positron emission tomography (PET) in 24 patients with acute (AVS, duration <1 month, n=11) or persistent (PVS, duration >1 month, n=13) vegetative state (VS) following prolonged anoxia due to cardiorespiratory arrest. After a follow-up period of twelve months, 8 patients had died, 13 remained in a permanent vegetative state and three showed moderate improvement of consciousness, without however regaining independence for activities of daily life. As expected, overall glucose utilization (CMRglc) was significantly reduced in VS in comparison to age matched controls. Infratentorial structures showed a less distinct hypometabolism. Differences in metabolic rates between patients who died or remained in a PVS were small and insignificant and probably reflect different age structures of the two groups. A statistically significant correlation between the degree of evoked potential or EEG alterations in VS and the reduction of global or regional cortical metabolic rates for glucose could not be established. Cortical metabolic rates in patients with PVS were significantly reduced when compared to patients studied in AVS (p<0.05 for all cortical regions of interest except the frontal lobe). This phenomenon reflects the progressive loss of residual cortical function following anoxic brain injury that corresponds to the neuropathological findings of progressive Wallerian and transsynaptic degeneration as sequelae of anoxic brain injury in PVS.

Chronic multifocal neuropathy with persistent conduction block (Lewis-Sumner syndrome). A clinico-morphologic study of two further cases with review of the literature.

We present data of 2 patients suffering from chronic motor-sensory multifocal neuropathy with persistent conduction block. The first case concerns a 9-year follow-up of a female, aged 24 years at onset with persistent multiple conduction blocks and a tendency towards generalization of clinical deficits. Eight years after onset sural nerve biopsy revealed extreme interfascicular variations of de/remyelination, onion bulb formation, fiber loss, edema, and proliferation of basal lamina of endoneurial capillaries. Serum antibodies against GM1 gangliosides were not detected. The second case, a 29-year-old Yemenitic male with a 5-year history, exhibited conduction blocks in motor and sensory fibers, and a normal sural nerve biopsy. Our results are discussed with respect to those of some 30 cases individually reported in the literature.

Sural nerve biopsy findings in leprosy: a qualitative and quantitative light and electron microscope study in 4 treated cases of the lepromatous spectrum.

Reports on biopsy findings in multifascicular nerves in lepromatous leprosy (LL) are rare and detailed morphometrical data are not available. In a case of early LL with normal electrodiagnostic findings in sural nerve, the present study revealed marked segmental de- and remyelination concomitant with the sequelae of considerable Wallerian degeneration of preferentially small myelinated fibers (MF) in spite of a normal number/nerve and density/mm2. Segmental de- and remyelination of several consecutive internodes in teased fibers suggests continuous bacterial spread via Schwann cells. In 2 more advanced LL-cases, nervous parenchyme was severely reduced, in a borderline lepromatous (BL) case obviously in part caused by cell infiltrates and granulomata. Distinct fascicle differences in MF-involvement were demonstrated by coefficients of variation of MF/mm2 and teased fiber preparations in LL, consistent with the hypothesis of initial focal spread of bacteria. Numbers and densities of endoneurial vessels were increased only in the later stages of LL. Enlargement of endoneurial area, due to different factors, was encountered except for the most severe LL-case with extensive endoneurial collagenization. Morphometric results were compared with those of other neuropathies. Intact and degenerating bacteria mostly in phagosomes of Schwann cells associated with unmyelinated axons and in macrophages were seen only in the early LL- and the BL-case. Sparse mononuclear cell infiltrates and small focal necrosis, present even in LL-cases, underline the complex pathogenesis of nerve fiber involvement.

[The MR tomographic diagnosis of intra- and paraspinal abscesses]. / Kernspintomographische Diagnostik intra- und paraspinaler Abszesse.

PURPOSE: Analysis of the MRT signals and their extent from intra- and paraspinal abscesses with reference to predisposing factors, their causes and localisation. PATIENTS AND METHODS: The histories and MRT findings in 34 Patients with intra- and paraspinal abscesses were evaluated retrospectively. Most of the patients (24/34) were older than 50 years. A second peak was below 30 years. RESULTS: 27/34 patients had some underlying disease which predisposed to infection, e.g., diabetes mellitus. The most common causal organisms were Staph. aureus (53%) and streptococci (15%). In 23/34 cases (68%), the abscesses were in the thoraco-lumbar or lumbar region, while only 6/34 occurred in the upper two-thirds of the thoracic spine and only 5/34 in the cervical region. In only 8/34 was the abscess confined to two vertebral lengths; in the remaining patients it was much more extensive. Intraspinal abscesses were about twice as large as the vertebral components and 1.5 times greater than paravertebral abscesses. The age of the abscesses could be estimated approximately from the signals. CONCLUSIONS: Contrast enhanced MRT permits detailed analysis of the compartments and exact estimation of the extent of the lesions and permits accurate monitoring of treatment.

Bell's palsy: electrodiagnostics are not indicative of cerebrospinal fluid abnormalities.

Electrodiagnostic testing (electromyography, electroneuronography, and blink reflex) and cerebrospinal fluid (CSF) examination (cell count, immunoglobulins, and antigen-specific intrathecal immunoglobulin G synthesis against herpes simplex virus, varicella zoster virus, cytomegalovirus, and Borrelia burgdorferi sensu latu) were performed in 56 patients with Bell's palsy. The CSF was normal in 45 patients and abnormal in 11 patients. Acute borreliosis was the most common specific pathological CSF finding (4 of 11). Electromyography revealed abolished volitional activity in 22% of patients with normal CSF and in 36% with pathological CSF. Electroneuronographic tests with an amplitude decrease of more than 90% on the affected side or abolished responses were found in 20% of patients with normal CSF and in 18% with pathological CSF. Abolished orbicularis oculi reflexes were seen in 67% of patients with normal CSF and in 82% with pathological CSF Concerning electrodiagnostic testing, no statistically significant difference between patients with normal and abnormal CSF was found, so we conclude that electrodiagnostic testing has no indicative value for abnormal CSF in Bell's palsy.

Long-term results of carpal tunnel decompression. Assessment of 60 cases.

The long-term prognosis of 60 patients operated on for carpal tunnel syndrome has been assessed in a prospective study with a median follow-up period of 5.5 years (range 2-11 years). Analysis of motor, sensory, trophic, and electrodiagnostic findings and assessment of pain were performed pre- and post-operatively using a standardized grading system. The results were generally favourable with a variable degree of improvement in 86% of cases. Statistical evaluation using multiple Dunn-Rankin tests revealed pain to be the most prominent pre-operative finding. On post-operative re-examination, pain was found to be improved to a significantly greater extent than any other variable. Analysis of several potential prognostic factors showed that pain lasting for more than 5 years prior to surgery indicates a poor prognosis. Only patients with diabetes mellitus exhibited a trend toward less pain relief.

The use of somatosensory evoked responses in carotid surgery for monitoring brain function.

One of the problems in carotid surgery is the intra-operative detection of brain ischemia. None of the methods applied so far have been successful. Experience has shown that the results were unsatisfactory. Experimental studies have detected a strong correlation between regional cerebral blood flow and somatosensory evoked potentials. At the Surgical Clinic of the University of Cologne, 60 patients had continuous intraoperative monitoring of somatosensory evoked responses during carotid endarterectomy. Our previous experiences suggest that monitoring of somatosensory evoked responses during carotid endarterectomy provides a suitable method for the registration of brain function. Furthermore it is possible to pinpoint the causes of ischemia and to immediately begin treatment based on the causes of ischemia.

[Hypokalemic paralysis in furosemide therapy and simultaneous laxative abuse]. / Dyskaliämische Lähmung bei Furosemidtherapie und gleichzeitigem Laxanzienabusus.

BACKGROUND: Clinical signs of hypokalemia are not directly related to the extent of the electrolyte imbalance, and therefore monosymptomatic cases may be observed. CASE REPORT: Following an acute gastroenteritis with considerable diarrhea, a 47-year-old male patient was admitted to hospital for progressive painful paraparesis. Upon admission, the patient complained of painful paresthesias in both legs, and a moderate flaccid paraparesis with widespread fasciculations and loss of leg tendon reflexes was found. Serum potassium level on admission was 1.7 mmol/l. Other signs of hypokalemia were absent, and the ECGs showed a slow sinus rhythm without disturbances of de- or repolarisation or cardiac arrhythmias. Hypokalemic paralysis was diagnosed and was considered to be primarily drug-induced, as the patient had a history of laxative abuse and was on a continuous medication with furosemide (80 mg/d) without regular assessment of serum electrolytes. The additional electrolyte loss following the gastroenteritis precipitated the development of clinical signs of hypokalemia. In parallel to the rise in serum potassium levels, both painful paresthesias and muscle weakness disappeared, and electromyography documented the amelioration of the myopathic syndrome. CONCLUSION: The prominent clinical symptom of hypokalemia was a dyskalemic paralysis in the absence of other sequelae of electrolyte imbalance, such as cardiac arrhythmias or vegetative disturbances.