Evidence-based review of therapeutic plasma exchange in neurological disorders.

Several neurologic disorders have been treated with therapeutic plasma exchange (TPE). Case reports, case series, and clinical trials have published results regarding the outcomes in such patients. The data gathered have been used to formulate evidence-based guidelines, which can be used to guide therapy in patients with these neurological disorders. Adequately designed and powered randomized controlled trials have proven the efficacy of TPE in some disease entities, while other diseases are lacking such data. In the latter, decisions for the use of TPE must be made using the limited published data available. In this review, we discuss the published evidence regarding the use of TPE in neurological disorders, focusing on the most recent guidelines published by the American Society of Apheresis in 2010 and the American Academy of Neurology in 2011.

Fatal Guillain-Barré syndrome.

Fourteen of 320 patients (4%) admitted with Guillain-Barré syndrome (GBS) died as a direct result of the illness. Deaths most commonly resulted from ventilator-associated pneumonia. In comparison with 101 other patients with severe GBS admitted to the intensive care unit, the patients who died were older (p = 0.006) and more likely to have underlying pulmonary disease (p = 0.004). In a specialized center, the primary event leading to death in GBS was ventilator-associated pulmonary infection, predominantly in elderly patients with significant comorbidity.

Acute renal failure due to severe Landry-Guillain-Barré syndrome.

BACKGROUND: Blood urea nitrogen (BUN) >60 mg/dl has been reported to occur commonly in patient's with severe Landry-Guillain-Barré syndrome. AIMS: To find out the cause for this high BUN we compared the renal function tests of 30 consecutive cases with severe Landry-Guillain-Barré syndrome to those of 30 controls. RESULTS: Acute renal failure occurred in seven patients with Landry-Guillain-Barré syndrome and none of the control group. Acute renal failure was found more in cases with Landry-Guillain-Barré syndrome compared to controls (P=0.0049). Six out of seven cases with Landry-Guillain-Barré syndrome and acute renal failure had dysautonomia and became oliguric while being in a hypotensive state. Of 30 patients with Landry-Guillain-Barré syndrome seven cases died. From eight patients with dysautonomia six cases who had acute renal failure died. The mortality rate was higher in cases with dysautonomia and acute renal failure (P = 0.0001 and 0.00001, respectively). Interestingly no glomerular disease was found. CONCLUSION: In conclusion acute renal failure can occur commonly in cases with severe Landry-Guillain-Barré syndrome particularly in those with dysautonomia, causing high mortality.

[Guillain-Barré syndrome in the adult: therapeutic aspects]. / Syndrome de Guillain-Barré de l'adulte: aspects thérapeutiques.

The Guillain-Barré syndrome is the most common cause of acute flaccid paralysis. The current mortality is 5%, while severe motor sequelae persist after one year in 10%. Multidisciplinary teams, trained in all specific treatments, are required to treat these patients. Oral and intravenous steroids have proved ineffective. Two large randomized clinical trials comparing plasma exchange (PE) with no treatment have shown a short-term and a 1-year benefit. The appropriate number of exchanges and the indications are now more precisely known. In the mild form (walking possible) patients should receive two PEs; a further two exchanges should be done in the event of deterioration or in advanced forms (loss of walking ability, mechanical ventilation). More exchanges are not beneficial. Recently two new randomized trials have produced evidence that intravenous immunoglobulin (IVIg) (0.4 g/kg/day for 5 days) was as effective as 5 PEs in advanced forms. The combination of PE with IVIg did not confer a significant advantage but increased the costs and risks. In advanced forms the choice between PE and IVIg depends on the contraindications for each treatment.

Axonal pathology in Japanese Guillain-Barré syndrome: a study of 15 autopsied cases.

We assessed the frequency and extent of axonal involvement in the ventral spinal roots in 15 Japanese autopsied patients with Guillain-Barré syndrome (GBS). Teased-fiber preparation revealed that five had predominantly axonal pathology with minimal segmental demyelination, seven had predominantly segmental demyelination with minimal axonal changes, two patients showed a mixture of both conditions, and one patient did not show any particular pathologic changes. We confirmed axon loss by immunohistochemical analysis of high-molecular-weight neurofilament protein. Macrophage invasion was a prominent feature in nerves with predominantly axonal changes. Two patients with severe axonal involvement and prolonged clinical courses exhibited motor neuron loss with astrogliosis in the ventral horns. These results suggest that autopsy-verified axonal involvement is more frequent among Japanese GBS patients than in Caucasian populations but less frequent than that reported from northern China.

Appropriate number of plasma exchanges in Guillain-Barré syndrome. The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome.

Plasma exchange (PE) is the standard treatment in Guillain-Barré syndrome (GBS) patients who have lost the ability to walk. The effect of exchanges before this stage and the optimal number of exchanges for the other patients are still unknown. We randomized 556 GBS patients according to severity and number of exchanges as follows: Zero versus 2 PEs for patients who could walk-with or without aid-but not run, or who could stand up unaided (mild group); 2 versus 4 PEs for patients who could not stand up unaided (moderate group); and 4 versus 6 PEs for mechanically ventilated patients (severe group). In the mild group, 2 PEs were more effective than none for time to onset of motor recovery (median, 4 vs 8 days, respectively). In the moderate group, 4 PEs were more beneficial than 2 for time to walk with assistance (median, 20 vs 24 days) and for 1-year full muscle-strength recovery rate (64% vs 46%). Six PEs were no more beneficial than 4 in the severe cases. Patients with mild GBS on admission should receive 2 PEs. Patients with moderate and severe forms should benefit from 2 further exchanges.

Síndrome de Guillain-Barré: evoluçäo do tratamento intensivo de 26 pacientes no período de 1984 a 1995 / Guillain-Barré syndrome: evolution of intensive care treament of 26 patients from 1984 to 1995

Objetivo: Avaliar a evolucao do tratamento da Sindrome de Guillain-BarT (SGB) em pacientes internados em terapia intensiva. Local: Unidade medico-cirurgica do Centro de Tratamento Intensivo do Hospital Universitario Clementino Fraga Filho da Universidade Federal do Rio de Janeiro. Tipo de Estudo: Coorte de insercao (revisao de prontuarios) de todos os pacientes internados com SGB no periodo de 1984 a 1995. Dados clinicos, o criterio diagnostico, tratamento efetuado e complicacoes foram coletados e analisados. O ponto final do estudo foi a evolutpo hospitalar (altas ou obitos). A analise estatistica: Teste de Fisher exato. Um valor de < 0,05 foi considerado estatisticamente significativo. Principais resultados: Foram avaliados 26 pacientes com SGB. Dividindo os pacientes em dois periodos: de 1984 a 1990 (Grupo 1 - 14 pacientes) e de 1991 a 1995 (Grupo 2 - 12 pacientes) observamos que: a) no primeiro perfodo os pacientes apresentaram mais complicacoes cardiovasculares relacionadas a disautonomia - bradicardia, assistolia, hipo e hipertensao (12/14 - 85,7 por cento vs. 5/12 - 41,6 por cento; p =, 0,02); b) no segundo os pacientes utilizaram mais plasmaferese, imunoglobulinas ou liquorferese (11/12 - 91,6 por cento vs. 1/14 - 7,1 por cento; p < 0,001); c) a letalidade no primeiro periodo foi de 11/14 - 78,6 por cento; jß no segundo periodo npo foram observados =bitos (p < 0,001); d) o tipo de assistencia ventilatoria empregada foi diferente - no primeiro perfodo utilizaram-se protese cicladas a pressao e a volume (14/14 - 100 por cento) enquanto que no segundo periodo a maioria fez uso de proteses microprocessadas (9/11 - 81,8 por cento) - p < 0,01; e) a incidencia de sepse, choque septico, hiperglicemia, pneumotorax, atelectasia e sindrome do desconforto respiratorio agudo foram similares nos dois periodos. Conclusoes: O tratamento da SGB com medidas imunomodulatorias e utilizando-se assistencia ventilatoria atraves de respiradores microprocessados sao extremamente eficazes, diminuindo as complicacoes cardiovasculares da disautonomia e a letalidade.

Assessment of Guillain-Barré syndrome mortality and morbidity in the United States: implications for acute flaccid paralysis surveillance.

To estimate age-specific incidences and assess the national morbidity and mortality burden for Guillain-Barre syndrome (GBS) in the United States, a national hospital discharge database compiled by the Commission on Professional and Hospital Activities (CPHA) and national death certificate data reported to the National Vital Statistics System were reviewed. During 1985-1991, 10,453 patients with GBS were discharged from CPHA-participating hospitals (estimated annual incidence, 3.0/100,000 population). The age-specific incidence of GBS increased with age from 1.5/100,000 in persons <15 years old to 8.6/100,000 in persons 70-79 years old. The total estimated number of GBS-related deaths from 1985 through 1990 was 3770 (95% confidence interval, 3506-4034), for an average of 628 GBS deaths per year. These rates suggest that the proposed national surveillance system for acute flaccid paralysis should capture at a minimum the 796 GBS cases in persons <15 years old. GBS remains a significant health burden among older adults in the United States, with a marked increase in risk after age 40.

Guillain-Barré syndrome.

The leading cause of acute neuromuscular weakness in the developed world is Guillain-Barré syndrome (GBS). Mortality rates vary widely. This article discusses the care and management of patients with GBS, with an emphasis on those patients who require admission to an intensive care unit.

Do corticosteroids influence the disease course or mortality in Guillain-Barre' syndrome?

Forty-six patients of Guillain-Barre' Syndrome were randomized to receive either prednisolone (40 mg daily for 2 weeks and then tapered off) or placebo. The patients were followed up for 6 months and were assessed on an objective scale of disability. The improvement in mean disability grade was significantly better at 2 weeks and 4 weeks in the placebo group as compared to those who received corticosteroids. The difference persisted at 24 weeks, but was statistically insignificant. A greater proportion of patients in the placebo group had improved by at least 1 disability grade at all points of time. The group of patients treated with steroids took twice as long to improve by 1 disability grade as compared to those in the placebo group. At 6 months, 41.7% of the patients in the steroid group had recovered almost completely (good outcome) as compared to 54.5% of the patients is the placebo group. Corticosteroids, therefore, do not appear to benefit GBS patients, and may in fact, delay the recovery from acute illness.

Campylobacter jejuni infection and Guillain-Barré syndrome.

BACKGROUND: Although infection with Campylobacter jejuni is recognized as a common antecedent of the Guillain-Barré syndrome, the clinical and epidemiologic features of this association are not well understood. METHODS: We performed a prospective case-control study in a cohort of patients with Guillain-Barré syndrome (96 patients) or Miller Fisher syndrome (7 patients) who were admitted to hospitals throughout England and Wales between November 1992 and April 1994. Bacteriologic and serologic techniques were used to diagnose preceding C. jejuni infection. RESULTS: There was evidence of recent C. jejuni infection in 26 percent of the patients with Guillain-Barré or Miller Fisher syndrome, as compared with 2 percent of household controls and 1 percent of age-matched hospital controls (P < 0.001). Of the 27 patients with C. jejuni infection, 19 (70 percent) reported having had a diarrheal illness within 12 weeks before the onset of the neurologic illness. No specific serotypes were associated with Guillain-Barré syndrome. C. jejuni infection was slightly more common in men (P = 0.14) and was more likely to be associated with a pure motor syndrome and a slower recovery (P = 0.03). The patients with preceding C. jejuni infection were more likely to have acute axonal neuropathy or axonal degeneration in association with acute inflammatory demyelinating polyradiculoneuropathy, and they had greater disability after one year (P = 0.02). C. jejuni infection was significantly associated with a poor outcome even after correction for other factors associated with a poor prognosis. CONCLUSIONS: Infection with C. jejuni often precedes the Guillain-Barré syndrome and is associated with axonal degeneration, slow recovery, and severe residual disability.

Management and outcome of severe Guillain-Barré syndrome.

Seventy-nine patients with Guillain-Barré syndrome admitted to a neurological intensive therapy unit (ITU) between 1985 and 1992 were studied retrospectively. The mean age was 49.8 years (range 16-86) and the time between the first neurological symptom and admission to ITU was 10.2 days (0-62). Admission was precipitated by a combination of respiratory failure requiring ventilatory support (73.4%), bulbar weakness (57.0%), autonomic features (11.4%) and general medical factors (10.1%). Specific treatments included plasma exchange (65.8%), intravenous immunoglobulin (13.9%) and methylprednisolone/placebo (12.7%). Significant complications included lower respiratory tract infections (45.6%), hyponatraemia (25.3%), dysautonomia (19.0%), urinary tract infection (12.7%) and cognitive disturbances (8.9%). Four patients (5.1%) died during the acute illness. Duration of nadir correlated with duration of ventilation, duration of ITU stay and outcomes at 3 months, 6 months and 1 year. However, time to nadir, an indicator of rapidity of deterioration, did not correlate with any outcome. The low mortality in this series of acutely ill and severely disabled patients suggests that specialized intensive therapy units continue to have an important role in the management of acutely ill patients with Guillain-Barré syndrome.

Critically ill Guillain Barre' syndrome.

Among the 153 patients fulfilling NINDS criteria for Guillain Barre' Syndrome (GBS) seen over 5.5 yrs, there were 47 (M:F 38.9) critically ill patients (age range 4 to 60 years). Antecedent event was recorded in 25 patients and the peak deficit was attained over a mean period of 9.5 days. Besides severe motor paralysis other salient features were: bulbar paralysis--42, sensory symptoms or signs--21, dysautonomia 31 and requirement for ventilatory assistance 45. CSF protein was raised in 63% cases. All the 17 patients who underwent electromyography had abnormalities of nerve conduction paramentes. Mean stay on the ventilator was 29.6 days and was not influenced by corticosteroid. Complications were frequent: pulmonary and urinary tract infection, dysautonomia, electrolyte disturbances, haemetmesis, bleeding from tracheostomy site and hepatic and renal failure. Mortality in steroids treated group (13/27) and the conservatively managed group (5/20) did not differ significantly. No discriminant factor emerged between survivors and non-survivors. Age and sex of the patients, presence of antecedent event, onset to peak interval and CSF protein level did not predict the need for ventilatory assistance, although these patients at admission had more frequent weakness of facial, bulbar, trunk, neck and proximal muscles of upper limbs and autonomic disturbances. Course of GBS remains unpredictable at the onset of the disease, warrants close supervision and meticulous supportive care and remains a therapeutic challenge.

Prognostic factors in Guillain-Barre' syndrome.

Prospective prognostic evaluation of various clinical and investigative parameters in 24 patients of Guillain-Barre' syndrome revealed poor prognosis in patients having rapid onset of illness, severe degree of paralysis and muscle wasting, prolonged period of peak paralysis lasting for more than 2 weeks and delayed onset of recovery not commencing within 3 weeks from the onset of weakness. Bulbar paralysis and respiratory involvement adversely affected the outcome. Electrodiagnostic evidence of fibrillation, polyphasic action potentials and markedly reduced amplitude of motor action potentials were indicative of poor prognosis. However, age and sex of the patients, severity of sensory loss, sphincteric disturbances, CSF findings and nerve conduction velocities did not significantly affect the outcome. Autonomic dysfunctions were noticed in 66.6% cases but were mostly mild and transient, and did not affect the long-term results.

Síndrome de Guillain-Barré. Experiencia en la Fundación Santa Fe de Bogotá

Se presenta el comportamiento clínico de los pacientes con síndrome de Guillain-Barré hospitalizados durante los años 1983 a 1991. Se observó el antecedente de un proceso infeccioso reciente en 14 casos (56 por ciento). Todos los pacientes presentaron debilidad de por lo menos dos extremidades y compromiso de pares craneanos en 12 casos (48 por ciento), de los cuales se afectó con mayor frecuencia el VII par (44 por ciento). El estudio electrofisiológico mostró una sensibilidad de 89 por ciento. El promedio de estancia hospitalaria fue de 29 días. El 24 por ciento requirió ventilación mecámica y la mortalidad fue de 8 por ciento. Se observó recuperación hasta llevar una vida independiente en 92 por ciento de los casos.

Plasma exchange treatment and prognosis of Guillain-Barré syndrome.

A retrospective analysis of case notes was undertaken for 52 patients presenting to Guy's Hospital with Guillain-Barré syndrome between August 1987 and September 1990. Twelve months after onset 61 per cent of patients had recovered completely, 35 per cent were still significantly disabled and two patients (4 per cent) had died. Forty-eight of the patients (92 per cent) were treated with plasma exchange. The frequency of morbidity related to this treatment was low, and plasma exchange was not responsible for either of the deaths. Outcome for all patients treated with plasma exchange between January 1985 and September 1990 was compared with that of 64 patients with Guillain-Barré syndrome seen in 1983 and 1984 who were not treated in this way. Time to walking unaided was significantly better (median 58 days compared with 86 days, p = 0.031), as was the median duration of ventilation (16.5 compared with 36 days, p = 0.004). Factors which had been found to predict an adverse outcome in previous studies (requirement for ventilation, age over 40 years, time to becoming bedbound less than 4 days, and small distally evoked abductor pollicis brevis muscle action potential) were not significantly associated with a poor prognosis in this study. The features associated with persisting disability were time to improvement more than 21 days, preceding diarrhoea and older age.

Guillain-Barré syndrome, pregnancy, and plasmapheresis.

The case of Guillain-Barré syndrome (acute inflammatory polyradiculoneuritis) in pregnancy reported here brings the total reported number to 35. The patient had plasmapheresis with no maternal or fetal complications. She did not require mechanical ventilation. This is only the fifth reported case of Guillain-Barré syndrome in the gravid patient involving plasmapheresis. There have been no controlled studies assessing the safety of plasmapheresis in the pregnant patient with the disorder. The possible risks of the procedure need to be weighed against the high maternal mortality rate in such a patient.