[Beware of unconspicious cerebro spinal fluid]. / Gefahrenquelle unauffälliger Liquorstatus.

Nowadays with a lot of diagnostic facilities it is also difficult to find the right diagnose of herpes encephalitis. The aim of this case report was to give an overview about strategies of diagnostic and therapy of the herpes encephalitis.

[Neuroborreliosis--a disease with many faces]. / Die Neuroborreliose - eine Erkrankung mit vielen Gesichtern.

Borreliosis has been widely recognized in Switzerland and is often used in unclear cases with non-specific symptoms. Two illustrative cases should emphasize the current options for diagnosis and therapy of neuroborreliosis. The keystones of the diagnostic instruments are a history with typical symptoms and analysis of the cerebrospinal fluid with determination of appropriate antibodies. Therapy deals with ceftriaxon with intravenous and doxycyclin with oral application making ambulatory treatment possible.

Carboplatin-Induced Bilateral Papilledema: A Case Report.

We report on a patient with carboplatin-induced bilateral papilledema, as it was described in the 1970s for cisplatin. Loss of visual accuracy up to full blindness, often loss of color vision and scotomas can be seen as a result of cortical blindness, macula degeneration, retrobulbar neuritis and papilledema. These symptoms are mostly unilateral and initially mild, so that more chemotherapy is given before the diagnosis is made. The symptoms are usually reversible within weeks to months after cessation of the platinum treatment. The therapeutic strategy is stopping the platinum treatment. In addition the empiric use of corticosteroids is suggested.

[Ganglioneuroma of the thoracic cavity in a child: case report]. / Ganglioneurom grudnog kosa kod deteta: prikaz slucaja.

INTRODUCTION: Ganglioneuromas are benign tumours that originate from the sympathetic chain. These kinds of tumours are most frequently benign neurogen tumours in childhood. Most often they are localized in the paravertebral area of posterior mediastinum. The tumour usually shows slow growth and patients are in most cases asymptomatic. These tumours are well encapsulated and surgical excision results in cure. CASE REPORT: We report a case of a 9 year-old boy with subpleural ganglioneuroma. When the child was admitted in hospital he had a pathological auscultatory pulmonary finding. The tumour was revealed by radiological examination during respiratory infection. Preoperative differential diagnosis included a number of intrathoracic lesions in the area of posterior mediastinum. The patient was operated with success. CONCLUSION: It is not clear if coughing was the only symptom of respiratory infection or a sign of intrapulmonary compression of the tumours mass. Intraoperative findings confirmed that the tumour was extirpated on time. Pulmonary parenchyma was without damage.

Membranoproliferative glomerulonephritis in two siblings: report and literature review.

There is evidence of a genetic basis in some cases of idiopathic membranoproliferative glomerulonephritis (MPGN) types I and III, particularly those occurring in families. The clinical and morphological features and disease course in two siblings with MPGN are described. In the male sibling, both clinical and morphological features as well as serum complement profile suggested type I MPGN; electron microscopy appearance in the female sibling was consistent with type III MPGN. Both patients had treatment-resistant nephrotic syndrome which evolved into renal insufficiency in the girl. No hereditary complement deficiencies were found in siblings or their parents. Both children exhibited HLA-A24; -B27, w4; -DR11, 52; -DQ3 antigens. Between 1981 and 1996, 18 patients from eight families with unequivocal diagnosis of MPGN I or III had been described. The mode of inheritance appeared to be autosomal dominant or X-linked in four of these families. In 11 patients, including our 2, in whom HLA typing was performed, eight had the HLA-A2 antigen. Similarities and discrepancies regarding clinical and morphological features and outcomes were evident in these intrafamilial cases, suggesting either a similar genetic background or a multigenic origin of MPGN. The familial occurrence of the MPGN, highlighted by our report, supports the concept that genetically determined factors may be involved in the pathogenesis of the disease.

Bladder neck incompetence in patients with spinal cord injury: significance of sympathetic skin response.

PURPOSE: We investigated whether recording the perineal sympathetic skin response, which reflects the sympathetic function of the thoracolumbar spinal cord, represents a reliable and accurate diagnostic tool for assessing bladder neck competence and incompetence. MATERIALS AND METHODS: We compared the sympathetic skin response recorded from the hand, foot and perineal skin with urodynamic findings in 90 patients with neurogenic bladder dysfunction, including 66 with spinal cord injury and 24 with cauda equina lesions. RESULTS: Video urodynamics revealed an incompetent bladder neck in 11 of 32 patients (34%) with complete and 7 of 34 (21%) with incomplete spinal cord injury but in only 1 of 24 (4%) with the conus-cauda equina syndrome. This association significantly correlated with the lesion level at T10 to L2 in 12 of 26 cases (46%) as well as with the loss of perineal but preserved hand and foot sympathetic skin response in 13 of 18 (72%). CONCLUSIONS: Recording the perineal sympathetic skin response in addition to that of the hand and foot represents a sensitive diagnostic tool for assessing sympathetic nerve function within the thoracolumbar spinal cord. It is of diagnostic value for evaluating neurogenic bladder neck incompetence in spinal cord injured patients.

[Pain therapy after spinal cord lesion]. / Schmerztherapie nach Rückenmarkläsion.

An important part of patients with spinal cord disorder suffers chronic pain. Especially the neuropathic pain syndrome is a problem. The pain lost his function of a protective warning sign, it can reduce the quality of life dramatically. We show the diagnosis and pharmacotherapy in neuropathic pain syndromes in patients with spinal cord disorder.

[Congenital postero-lateral right diaphragmatic hernia--case report]. / Kongenitalna posterolateralna desna dijafragmalna hernija--prikaz slucaja.

OBJECTIVE: There are three types of congenital diaphragmatic hernias (DH): posterolateral (Bochdalek) diaphragmatic hernia, subcostosternal (Morgagni's) hernia and esophageal hiatal hernia. The posterolateral (Bochdalek) diaphragmatic hernia is the result of a congenital diaphragmatic defect in the posterior costal part of the diaphragm in the region of 10th and 11th ribs, which allows free communication between the thoracic and abdominal cavity. The defect is usually found at the left side (90%) but may occur on the right side, where the liver often prevents detection. CASE REPORT: We present a 9-year-old girl with persistent cough during four weeks, vomiting and loss of appetite. She was admitted in hospital after one month ambulatory treatment without success. Chest x-ray showed an infiltrative shadow in basal lobe of the right lung and multiple radiolucencies over the right hemithorax. Irrigography confirmed presence of contrast in the right hemithorax (with herniation of colon in the right lung). The child had a restrictive type of pulmonary function and was operated after establishing the diagnosis of the disease. Posterolateral diaphragmatic defect was established intraoperatively with diameter 10 x 11 cm and with ascending colon prolapse, caecum appendix and part of colon transfersum. The child was in good condition after operation. Control X-ray was normal and the child has a normal pulmonary function on routine controls. COMMENT: The posterolateral right congenital DH is a rare diaphragmatic defect. The symptoms are noncharacteristic and patients with this disease can be without symptoms for a long period. The main tool for diagnosis of congenital DH is radiography, whereas treatment is surgical.

[Familial membranoproliferative glomerulonephritis]. / Familijarni membranoproliferacioni glomerulonefritis.

INTRODUCTION: Idiopathic membranoproliferative (mesangiocapillary) glomerulonephritis (MPGN) is a chronic, often progressive renal disease with variable clinical expression divided into three distinct morphological formes, now designated types I, II, and III, on the basis of immunofluorescent (IF) and ultrastructural appearances and complement profiles [1-5]. Several lines of evidences suggest a genetic basis for at least some cases of MPGN I and III. The extended haplotypes HLA-B8, DR3, SCO1, GLO2 were found to constitute 13% of the disease-associated haplotypes and 1% of control hyplotypes [8]. Significantly high percentage of those with MPGN I and III have inherited defects of the complement system [9]. Additional evidence for genetic factors is the rarity of the disease in blacks [10] and examples of MPGN occurring in families. The disease has been reported in siblings as well as in families with affected members spanning more than one generation [11-16]. Here we describe clinical and morphological features in two siblings affected by MPGN and present complement and HLA typing studies done in patients and their parents. A review of familial MPGN I and III cases reported between 1981 and 1996 is made, and genetic susceptibility factors for MPGN are discussed. SUBJECTS AND METHODS: Between 1976 and 1996 diagnosis of idiopathic MPGN was made in 24 patients, aged 516.5 years. The diagnosis was established after excluding systemic, liver and infectious disorders and malignant neoplasms. The MPGN type was confirmed by light microscopy, IF and electron microscopy studies of the renal biopsy tissues processed by standard techniques. One family with two siblings having MPGN was identified in our series. This family was examined for the presence of renal disease and an inherited complement defect. Laboratory evaluations of the patients and parents included complete urinalysis, serum protein, albumin, urea, creatinine and cholesterol levels and glomerular filtration rate (GFR) estimation. ANA, rheumatoid factors, cryoglobulins, immune complexes, HBV antigens and antibodies and anti-HCV antibodies were also determined. Haemolytic tests for CHSO (classical and alternative pathways) were carried out using standard techniques. The measurement of the various complement factors was carried out using a radial immunodiffusion technique with monospecific antisera (CIq, C2, C4, C3, C5, B, H). HLA-A, B, DR and DQ haplotypes were determined by microcytotoxicity assay of peripheral blood lymphocytes. RESULTS: Patient 1 (SC, male). Renal disease presented at the age of his five years with nephrotic syndrome resistant to corticosteroid treatment. Morphological features and serum complement profile suggested type I MPGN. Treatment consisted of alternate-day prednisone, followed by cyclosporine and then by cyclophosphamide. At the end of the follow-up lasting 5.5 years he had only moderate proteinuria. Patient 2 (MC, female). Proteinuria was revealed at the age of 3 years becoming progressive and leading to the nephrotic syndrome resistant to corticosteroids at the age of 6 years. Electronmicroscopy features were consistent with type III MPGN, although serum C3 and C4 levels remained normal all the time. The same treatment as in her brother was given but she remained persistently nephrotic and anaemic; hypertension developed when she was 6 years old and her renal function became to declaine at the age of 7.5 years. Detailed family studies failed to reveal any evidence of complement deficiencies or secondary cause of MPGN. Siblings had in common HLA-A24, B27, Bw4, DRI1, DRS2, and DQ3 antigens. DISCUSSION: In our patients clinical and morphological features are very similar and are consistent with diagnosis of MPGN, i.e. probably type I in the boy and type III in the girl. Although some extended haplotypes HLA-B8, DR3, SCO1, GLO2, were significantly more frequent (13%) than in controls (1%), and the patients with MPGN having this extended haplotype had

Does sacral posterior rhizotomy suppress autonomic hyper-reflexia in patients with spinal cord injury?

OBJECTIVE: To study the occurrence of autonomic hyper-reflexia (AHR) after intradural sacral posterior rhizotomy combined with intradural sacral anterior root stimulation, performed to manage the neurogenic hyper-reflexic bladder and to determine the pathophysiological basis of the uncontrolled hypertensive crisis after sacral de-afferentation. PATIENTS AND METHODS: Ten patients with spinal cord injury operated using Brindley's method between September 1990 and February 1994 were reviewed. Systematic continuous non-invasive recordings of cardiovascular variables (using a photoplethysmograph) were made during urodynamic recordings and the pre- and post-operative vesico-urethral and cardiovascular data compared. RESULTS: Nine of the 10 patients were examined using a new prototype measurement system; one woman refused the last urodynamic assessment. Eight of the nine patients who presented with AHR before operation still had the condition afterward. There was a marked elevation in systolic and diastolic blood pressure during the urodynamic examination in all eight patients, despite complete intra-operative de-afferentation of the bladder in five. The elevation of blood pressure started during the stimulation-induced bladder contractions and increased during voiding in all cases. Five patients showed a decrease in heart rate during the increase in blood pressure. However, in three patients the heart rate did not change or even sometimes slightly increased as the arterial blood pressure exceeded 160 mmHg, when the blood pressure and heart rate then increased together. CONCLUSIONS: These results confirm that even after complete sacral de-afferentation. AHR persisted in patients with spinal cord injury and always occurred during the stimulation-induced voiding phase. In cases of incomplete de-afferentation, small uninhibited bladder contractions without voiding occurred during the filling phase. The blood pressure then increased but never reached the value recorded during stimulation-induced micturition. Stimulation of afferents that enter the spinal cord by the thoracic and lumbar roots and that are not influenced by sacral rhizotomy could explain why AHR increases during urine flow. The distinct threshold of decreased heart rate by increasing blood pressure to > 160 mmHg focuses attention on the chronotropic influences of the sympathetic nerves in the heart by an exhausted baroreceptor reflex.

Botulinum A toxin as a treatment of detrusor-sphincter dyssynergia in patients with spinal cord injury: MRI controlled transperineal injections.

OBJECTIVES: To correlate clinical and urodynamic findings with MRI in patients with spinal cord injury and detrusor-sphincter dyssynergia who were consecutively treated with transperineal injections of botulinum-A toxin (BTX-A) under EMG control. METHODS: Six patients with spinal cord injury and upper motor neuron bladder dysfunction associated with detrusor-sphincter dyssynergia were prospectively analysed. One hundred international units (IU) BTX-A (Botox in 1 ml normal saline without preservative) diluted 1 to 1 with 1 ml gadopentetate were injected transperineally under EMG control. MRI was started immediately after needle withdrawal. RESULTS: In all six patients gadopentetate was located in the external urethral sphincter on MRI. In no patient did traces of gadopentetate appear in the perineal musculature located in the vicinity of the external urethral sphincter. No patient developed resistance to BTX-A. All patients showed an (ongoing) improvement of their voiding function after BTX-A injections. CONCLUSIONS: Transperineal injections of BTX-A under EMG control are efficient in the release or amelioration of lower urinary tract obstruction due to detrusor sphincter dyssynergia in patients with spinal cord injury. Despite well described methods, EMG of the external urethral sphincter is difficult and it is not possible to definitively exclude false recordings of the surrounding perineal musculature. By the use of MRI it was shown that both the EMG recordings and transperineal injection method are precise.

Recovery of bladder function in patients with acute spinal cord injury: significance of ASIA scores and somatosensory evoked potentials.

The significance of the ASIA (American Spinal Injury Association) scores and SSEP (somatosensory evoked potentials) recordings in predicting the recovery of bladder function was evaluated in 70 patients with acute, traumatic spinal cord injury (SCI). The patients were examined following admission to the rehabilitation centre (mean 10 days post-trauma) both clinically by the ASIA scores and electrophysiologically by tibial and pudendal SSEP recordings. The results of the initial examinations were related to the degree of recovery of bladder function of the patients assessed by urodynamic examination at the end of the rehabilitation programme (at least 6 months post-trauma). The recovery of somatic nerve function (external urethral sphincter function) involved in bladder function was correlated to both the initial ASIA scores and SSEP recordings (Spearman correlation, P < 0.001). The latter parameters, however, were not related to the outcome of autonomic nerve function (eg detrusor vesicae function) (Spearman correlation, P = 0.1). Therefore, the initial clinical and electrophysiological examinations are of value in assessment of the degree to which the patient will recover somatic nervous control of bladder function. However, these examinations are not indicative of urodynamic impairment. Therefore, urodynamic examination should be mandatory for the diagnostic assessment and therapeutical approach of bladder dysfunction in patients with acute SCI.

Assessment of autonomic dysreflexia in patients with spinal cord injury.

OBJECTIVES AND METHODS: To assess the impairment of supraspinal control over spinal sympathetic centres and the occurrence of autonomic dysreflexia in patients with spinal cord injury. Autonomic dysreflexia is caused by the disconnection of spinal sympathetic centres from supraspinal control and is characterised by paroxysmal hypertensive episodes caused by non-specific stimuli below the level of the lesion. Therefore, patients with spinal cord injury were examined clinically and by different techniques to assess the occurrence of autonomic dysreflexia and to relate disturbances of the sympathetic nervous system to episodes of autonomic dysreflexia. RESULTS: None of the paraplegic patients, but 59% (13/22) of tetraplegic patients (91% of the complete, 27% of the incomplete patients) presented signs of autonomic dysreflexia during urodynamic examination. Only 62% of the tetraplegic patients complained about symptoms of autonomic dysreflexia. Pathological sympathetic skin responses (SSRs) of the hands were related to signs of autonomic dysreflexia in 93% of cases. No patient with preserved SSR potentials of the hands and feet showed signs of autonomic dysreflexia, either clinically or during urodynamic examination. Ambulatory blood pressure measurements (ABPMs) indicated a loss of circadian blood pressure rhythm (sympathetic control) but preserved heart rate rhythm (parasympathetic regulation) only in patients with complete tetraplegia. Pathological ABPM recordings were seen in 70% of patients with symptoms of autonomic dysreflexia. CONCLUSIONS: The urodynamic examination was more sensitive in indicating signs of autonomic dysreflexia in patients with spinal cord injury, whereas SSR allowed the assessment of the degree of disconnection of the sympathetic spinal centres from supraspinal control. Using ABPM recordings the occurrence of episodes of autonomic dysreflexia over 24 hours and the effectiveness of therapeutical treatment can be assessed.

Posterior sacral rhizotomy and intradural anterior sacral root stimulation for treatment of the spastic bladder in spinal cord injured patients.

PURPOSE: The efficacy of intradural sacral posterior rhizotomy combined with intradural sacral anterior root stimulation in the treatment of the neurogenic hyperreflexic bladder was evaluated. MATERIALS AND METHODS: We reviewed 10 spinal cord injured patients who underwent surgery between September 1990 and February 1994. Bladder function was compared preoperatively and postoperatively. Intraoperative data on electrostimulation of the detrusor and striated muscles were analyzed. RESULTS: Stimulation of the anterior S3 and S4 roots was mostly used to empty the bladder (7 of 10 cases). Preoperative reflex incontinence disappeared in all patients postoperatively. Mean postoperative bladder capacity increases and mean postoperative post-void residual decreases were at least 340 ml. (p < 0.01) and 140 ml. (p < 0.01), respectively. Preoperative vesicorenal reflux disappeared in 2 and improved in 3 cases after sacral deafferentation. Autonomic hyperreflexia, which was present preoperatively in 6 patients, never disappeared but significantly improved after deafferentation. No major complications were noted postoperatively. CONCLUSIONS: Intradural sacral posterior rhizotomy combined with intradural sacral anterior root stimulation is a valuable method to treat the hyperreflexic bladder with incontinence resistant to conservative therapy in spinal cord injured patients. Autonomic hyperreflexia was decreased but not suppressed by posterior sacral rhizotomy.

Botulinum-A toxin as a treatment of detrusor-sphincter dyssynergia: a prospective study in 24 spinal cord injury patients.

PURPOSE: The paralytic effect of botulinum-A toxin injections on the external urethral sphincter was investigated prospectively in patients with neurogenic voiding disorders. MATERIALS AND METHODS: Transurethral versus transperineal botulinum-A toxin injections were performed in 24 spinal cord injury male patients with detrusor-sphincter dyssynergia and the respective efficacy was compared. RESULTS: In 21 of 24 patients detrusor-sphincter dyssynergia was significantly improved with a concomitant decrease in post-void residual volumes in most cases. Botulinum-A toxin effects lasted 3 to 9 months, making reinjections necessary. CONCLUSIONS: Although costly, botulinum-A toxin injections, which aim at suppressing detrusor-sphincter dyssynergia but not bladder neck dyssynergia, appear to be a valid alternative for patients who do not desire surgery or are unable to perform self-catheterization.

Determination of cathepsins B and H in sera and synovial fluids of patients with different joint diseases.

Synovial fluids and sera of patients with inflammatory and metabolic joint diseases contain different cysteine proteinases. The quantities of cathepsins B and H were determined by newly developed specific enzyme-linked immunoassay tests (ELISA), with detection limits of 0.5 microgram/l for cathepsin B and 3 micrograms/l for cathepsin H. The values of cathepsin B in normal sera ranged from 0.6 microgram/l to 2 micrograms/l, whereas in sera of patients with joint diseases they ranged from 1.7 micrograms/l to 18 micrograms/l. Cathepsin H was not found in sera (values below 3 micrograms/l), but was measurable in patients' synovial fluids. Patients with rheumatoid arthritis have on average the highest values of cathepsin B in synovial fluids, whereas patients with undifferentiated arthritis have the highest values of cathepsin H. The results show that cathepsins B and H are present in arthritic synovial fluids, where they may be implicated in destructive processes. There is yet no clear correlation between the quantity of each cathepsin released in synovia and the clinical diagnosis or the stage of the disease.

Kinetic study of rheumatoid factor influence on complement-mediated modulation of immune precipitation.

Human monoclonal IgM kappa rheumatoid factor PA (mRF) expressed various effects on complement-dependent modulation of ovalbumin/antiovalbumin lattice formation measured kinetically by laser light scattering (LLS): (1) in the absence of complement, mRF (44.2-177 U/ml) caused enhancement of LLS in a dose-dependent manner; (2) mRF partially prevented expression of complement-mediated inhibition of lattice formation and, at the same time, complement inhibits mRF-dependent LLS enhancement; (3) complement caused disruption of lattice-mRF bonds during solubilization of preformed lattice-mRF complex as demonstrated by specific mRF activity in supernatants. This effect is dependent on complement activity and on both complement and mRF concentrations; (4) ovalbumin/antiovalbumin complexes gradually lost the ability to react with mRF during the first 2 min of complement-mediated inhibition of lattice formation.

Study of complement effects on kinetics of immune precipitation.

Influence of complement on the kinetics of immune precipitation was investigated by using simple kinetic tests. In the early phase of immune precipitate (IPPT) formation, it was demonstrated that complement caused inhibition of precipitation only. After formation of a certain critical IPPT mass, alternative pathway-dependent enhancement of precipitation was observed, followed by solubilization of 'enhanced' IPPT and, at last, by solubilization of preformed IPPT. Contrary to inhibition and IPPT solubilization, enhancement of IPPT formation does not change IPPT ability to react with fresh complement.