Hou-Chang Chiu

Plasmapheresis in the Treatment of Ataxic Sensory Neuropathy Associated with Sjögren’s Syndrome

Sjögren’s syndrome (SS) is an important but poorly recognized cause of peripheral neuropathy. Several forms of peripheral nerve dysfunction occur, including trigeminal sensory neuropathy, mononeuropathy multiplex, distal sensorimotor polyneuropathy and pure sensory neuronopathy. The pathological findings vary and the definite treatment is not known. Here we present 4 cases of acute ataxic sensory polyneuropathy with SS, and the experience of treatment with plasmapheresis (PP). The 4 patients were all females; ages ranged from 30 to 58 years. All had prominent loss of kinesthetic and proprioceptive sensation. The course ranged from acute to subacute onset. Patients were treated with 5–9 sessions of PP. Two patients with initiation of treatment within 2 weeks of onset showed dramatic and sustained responses after PP, while the other 2 had no detectable effects. Our experience showed that PP should be considered in patients who present with sensory neuropathy associated with SS, and the treatment should be given as early as possible.

Double filtration plasmapheresis in myasthenia gravis - analysis of clinical efficacy and prognostic parameters

Objectives The aim of this study was to evaluate the efficacy of double filtration plasmapheresis (DFP) in the treatment of patients with myasthenia gravis (MG) and to analyze the possible prognostic factors related to responsiveness to DFP. Materials and methods‐ We treated 45 MG patients, 26 women and 19 men aged 21–72 years, with DFP for 5 consecutive sessions. All were affected by severe generalized or respiratory weakness with an Ossermans classification of group 2 or 3 and had not responded to previous treatments. Results‐ Thirty‐eight out of 45 patients (84%)) achieved significant improvements after DFP. The baseline MG score and removal rate for immunoglobulin G (IgG) were significantly higher in the patients with good response than in the other response groups. Poor responders were more likely to have thymoma and a longer interval among sessions of DFP. Better response in patients with age at onset of less than 40 years was associated with higher MG score. Serum concentration of all proteins tested fell as follows (mean ± SD): IgM, 88 ± 7%; IgA, 71 ± 11%; IgG, 59 ± 14%; globulin, 52±11%; AchRAb, 47±14%; and albumin, 27±10%. All the patients tolerated plasmapheresis well except for 2.2% who experienced hypotension. Conclusion‐ In this study, DFP was effective and safe in the treatment of patients with severe generalized MG. The factors correlating with the better clinical response were high MG score, a thymic pathology of non‐thymoma, daily apheresis, young age at onset, and high removal rate for IgG.

Experience of double filtration plasmapheresis in the treatment of Guillain-Barré syndrome

Therapeutic plasma exchange (TPE) is a standard treatment in Guillain‐Barré syndrome. TPE may require exogenous fluid for replacement of plasma and, depending on the equipment used, varying extracorporeal volumes. Potential adverse effects include allergic reaction, infection, and hypotension. From September 1993 to December 1997, we treated 16 patients with Guillain‐Barré syndrome by a newly developed method of automated double filtration plasmapheresis (DFPP). Patients (ten males and six females, age ranged from 16 to 73) suffering from acute ascending motor weakness and fulfilling the diagnostic criteria for GBS were chosen for DFPP. Each patient received at least five sessions of apheresis in 7 to 10 days and approximately 2.5 to 3.0 L of plasma was treated in each session. Patients were evaluated by disability grade according to a Hughes scale. The mean grade of disability was 3.62 at treatment and improved to 2.37 four weeks after the start of DFPP. The median time to grade 2 (walk without support) was 19 days. There were five patients (41.6%) in need of respirator support. The median time to weaning off the respirator was 9 days. Only two patients (12.5%) could not reach grade 2 at the end of 6 months. Our results were comparable to previously published results of TPE. We conclude that DFPP may be as effective as TPE in the treatment of GBS. J. Clin. Apheresis 14:126–129, 1999.

Double filtration plasmapheresis in the treatment of myasthenic crisis – analysis of prognostic factors and efficacy

Objectives– To examine the prognostic factors and outcome of myasthenia gravis (MG) patients in crisis with double filtration plasmapheresis (DFP) treatment. Material and methods– A total of 15 patients experienced 20 episodes of crisis during the study period. Plasmapheresis was carried out using a double filtration method. Demographic information, clinical features of crisis, and associated complications were analyzed. Results– The median duration of crisis was 9 days. Chest infection was the most common precipitant of crisis. Twelve out of the 20 episodes (60%) responded well to DFP and mechanical ventilation was discontinued after the third session of DFP in 8 of them. Three significant predictors for prolonged crisis were shorter intervals between the onset of MG and the first crisis (P=0.04), higher serum bicarbonate levels at baseline (P=0.03) and the thymic pathology of thymoma (P=0.03). Conclusion– DFP can ameliorate the profound weakness in crisis and seems to be a rational therapy for patients with myasthenic crisis.

High Titer of Anticardiolipin Antibody Is Associated with First-Ever Ischemic Stroke in Taiwan

Background and Purpose: The association between anticardiolipin antibody (aCL) and ischemic stroke is controversial, and there are few case-control studies of Asian populations. The aim of this study, therefore, was to determine whether aCL is an independent risk factor for ischemic stroke in Taiwanese patients over the age of 40 years. Methods: Both the IgG and IgM isotypes of aCL were measured in 273 patients (>40 years of age) hospitalized for first-ever ischemic stroke and in 181 non-stroke controls. Results were defined as: negative (<10 IgG phospholipid units [GPL] or <7.5 IgM phospholipid units [MPL]); low positive (10–20 GPL or 7.5–15 MPL); or, high positive (>20 GPL or >15 MPL). Odds ratios (OR) were estimated by logistic regression with adjustment for potential confounders. Results: A high positive IgG aCL was present in 4.4% of the stroke patients and 1.2% of the controls. Age- and sex-adjusted analysis showed a borderline association between a high positive level for aCL IgG titer and stroke, with an OR of 4.01 (95% CI 0.87–18.37; p = 0.0739). Final analysis, with adjustments for age, sex, hypertension, diabetes, tobacco smoking, atrial fibrillation, left ventricular hypertrophy and hyperlipidemia, revealed an OR of 5.25 (95% CI 1.06–25.89; p = 0.0419). Conclusions: The results of this study suggest that elevated titer of aCL IgG (>20 GPL) is associated with first-ever ischemic stroke in Taiwanese patients aged over 40 years. High positive aCL titer is related to ischemic stroke after adjustment for conventional cerebrovascular risk factors, indicating that it is probably an independent risk factor for ischemic stroke.

Predicting the course of myasthenic weakness following double filtration plasmapheresis.

Objective – To evaluate the clinical course of patients with myasthenia gravis (MG) up to 3 months after double filtration plasmapheresis (DFP).

The eight-year experience of plasmapheresis in patients with neurological diseases

Plasmapheresis (PP) removes component parts of plasma from patients, has been shown to be effective in the treatment of a variety of neurological autoimmune diseases including myasthenia gravis (MG), acute and chronic demyelinating polyneuropahty (AIDP and CIDP), polyneuropaties associated with inflammatory monoclonal gammopathy and myasthenic syndrome. The mechanism proposed for the actions of PP include removal of antibody, alloantibody, immune complexes, monoclonal protein, cytokines and antitoxin. According to the neurological report of American Academy of Neurology assessment of plasmapheresis in 1996, three diseases are based on class I evidence and with types A recommendation, Guillan-Barre Syndrome, CIDP and polyneuropathy with monoclonal gammopathies. Myasthenia gravis is class IV evidence with type C commendation. From November 1993 to December 2001, we treated 280 patients with various diseases by PP for a total of 421 courses. These included 148 cases of MG, 67 cases of AIDP or CIDP, 3 cases of multiple sclerosis and 2 cases of motor neuron disease. PP were carried out by and automated double filtration method (KM-8800, Kuraray or Plasauto-iQ, Asahi). The Plasmacure (Kuraray) or Plasmaflo AP05W (Asahi) were used as plasma separator and Evaflux 4A or 5A (Kuraray) or Cascadeflo AC 1770 (Asahi) as plasmafracitionater. Each course of treatment consists of 4 to 5 sessions of apheresis. The processed volume of plasma is one calculated plasma volume. All patients tolerated PP well although 3% of them experienced hypotension. Our experiences are summarized as follows. For MG patients, both DF and IA effectively ameliorate symptoms and signs of MG. IA removes acetylcholine receptor antibody more effectively than DF does, but clinical effects between these two methods are similar. Daily schedule seems more effective than alternately daily schedule. The optimal number of PP sessions for each course is four based on the clearance of acetylchoine receptor antibody. The factors correlating with better clinical response are high MG score, non-thymoma patients, younger age at onset, and higher removal rate for immunoglobulin. For patients with AIDP, the median time to grade 2 (walk without support) was 19 days and to waning off the respirator was 9 days. The Clinical efficacy of double filtration PP in the treatment of AIDP was comparable to that of plasma exchange. *** DIRECT SUPPORT *** A00RC003 00008

Increased intracranial resistance as reflected by low end diastolic velocity, high resistance index, and increased diameter of extracranial carotid arteries has better power than extracranial carotid atherosclerosis in predicting ischemic stroke in Chinese of Taiwan

86 Background: Peak systolic velocity (PSV) has been considered a good index of detecting high degree stenosis (>50%) of carotid arteries and has been used in combination with stenosis percentage to identify high risk individual for ischemic stroke (IS). Fewer studies focused on other parameters such as end diastolic velocity (EDV), flow volume (FV), resistance index (RI), and diameter (DIA). We aimed to compare the predictive power of various Duplex ultrasonography indices on ischemic stroke in Chinese. Methods: We conducted a case-control study on 251 first-ever IS patients (age≥40) and 242 age and sex matched outpatient controls. Duplex indices of spectral analysis and image were obtained. Percentage of stenosis, plaque score (PS), PSV, EDV, RI, DIA, and FV of right carotid arteries were included the analysis. Results: Parameters of common carotid artery (CCA) were used due to its stronger predictive power of IS risk than those of internal and external CA. Higher sensitivity and specificity for IS were demonstrated for each of EDV, RI and DIA than PS, stenosis percentage, PSV, and FV. Factor analysis extracted two factors out of 5 best indices, which were intracranial resistance factor (composed of EDV, RI, and DIA) and carotid atherosclerosis factor ( PS and stenosis percentage). Significantly high age-and-sex-adjusted odds ratios for IS appeared on those subjects with low EDV (OR=4.93, p=0.0001), high RI (OR=3.26, p=0.0001), large DIA (OR=2.82, p=0.0001), or positive PS (OR=2.51, p=0.0001). Higher odds ratios (OR=10.16, p=0.0000) were occurred in those subjects with low EDV, high RI, and large DIA combined. Conclusion: An intracranial resistance component may be described by low EDV, high RI, and large DIA. We found strong associations of IS risk with EDV, RI, and DIA separately or combined. This association was much stronger than that of PS or that of stenosis percentage, indicating the significance of intracranial resistance in the pathogenesis of ischemic stroke in Chinese.

Dilatation of common carotid artery is strongly associated with cerebral ischemic stroke with or without the presence of carotid atherosclerosis

P147 Background: Dilatation of common carotid artery (CCA) was related to age, sex, and body height in population studies. It was also considered a compensatory mechanism to carotid atherosclerotic stenosis. The present study examined the risk of CCA dilatation associated with ischemic stroke (IS) and its relations to carotid atherosclerosis, hypertension, hyperglycemia, fibrinogen, cholesterol, HDL-cholesterol (HDL-C), smoking, and alcohol consumption. Methods: A case-control study was carried on 251 first-ever IS patients (age≥40) excluding previous history of myocardial infarction and cancer and 242 non-stroke outpatients. Intraluminal diameter of middle portion of CCA, and plaque thickness in CCA, bulb, internal and external carotid arteries were measured. Information on hypertension and diabetes status and data of life-styles such as smoking and alcohol consumption were collected. Levels of fibrinogen, factor VIIIc, cholesterol, HDL-C and glucose were obtained. Results: CCA dilatation was a strong factor for IS (OR=4.13, P=0.0001). It was also associated with hypertension, hyperglycemia, smoking, alcohol consumption, low HDL-C, and high levels of fibrinogen, factor VIIIc, cholesterol, and plaque score. The association remained significant with or without each of the following conditions: hypertension (p=0.0001, p=0.0007), hyperglycemia (p=0.0446, p=0.0001), elevated fibrinogen (p=0.0104, p=0.0001) or factor VIIIc (p=0.2458, p=0.0001), hypercholesterolemia (p=0.0238, p=0.0001), decreased HDL-C (p=0.0012, p=0.0001) and presence of plaque score (p=0.0263, p=0.0003). Adjusting above risk factors, odds ratios of elevated diameter could associated with IS, before (OR=2.21, P=0.0066) and after (OR=6.63, p=0.0055) excluding subjects with plaque. Conclusion: Dilatation of CCA is a strong risk factor for IS. The fact the association remained significant without ultrasonic evidence of carotid plaque indicates that IS in Chinese involved a mechanism of active vasculopathy, not just a passive compensatory process to extracranial atherosclerosis.