Long-term outcome of Guillain-Barré syndrome

Objectives  

To investigate long-term neurological residua after Guillain-Barré syndrome (GBS) and to evaluate the predictive value of respiratory insufficiency during the acute stage of the disease.     

Long-term impact on work and private life after Guillain-Barré syndrome

OBJECTIVE: To determine the long-term impact of Guillain-Barré syndrome (GBS) on work and private life of patients and their partners. METHODS: Three to six years after the onset of GBS 150 patients who participated in the Dutch Guillain-Barré trial received a questionnaire specifically drafted for this study to survey their present psychosocial status. Furthermore, their present physical status was established. RESULTS: A total of 122 patients participated. Thirty-one percent showed moderate to serious physical residua after a functional assessment. Due to GBS, 38% of the patients who held a job had to change it, 44% altered their leisure activities, 37% of the patients did not function as well at home as before GBS and 39% reported a change in their partners' lives. Almost half of the patients still had negative comments on their present psychosocial situation. CONCLUSION: GBS has a serious long-term impact on the patients' work and private life and that of their partners.     

Guillain-Barré syndrome after a jellyfish sting

This is the case of a jellyfish sting associated with the rare development of Guillain-Barré syndrome. The patient, a 66-year-old woman, was stung by a jellyfish on the right thigh while swimming in the Atlantic Ocean, off Charleston, SC. Ten days later, she developed low back and right thigh pain followed by progressive numbness and weakness in all extremities. These symptoms reached their peak in 30 days and slowly began to improve. Initial neurologic examination showed areflexia, weakness, absent vibration and position sense, and hyperesthesia to pin and light touch in the mid to distal region of all four extremities. Serial electromyography and nerve conductions were consistent with an improving predominantly demyelinating polyneuropathy. Spinal fluid analysis showed no cells, elevated protein (108), gammaglobulin 6 (normal less than 5.4), and immunoglobulin G 8.2 (normal less than 6). The only treatment was gabapentin for neuropathy pain. The patient made an excellent recovery in less than 1 year with minimal residual numbness in both thumbs, index fingers, and middle toes.     

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is currently divided into the two major subtypes: acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). This review highlights relevant recent publications, particularly on the pathophysiology of AMAN. Molecular mimicry of the bacterial lipo-oligosaccharide by the human gangliosides is now considered an important cause of AMAN. Gangliosides GM1, GM1b, GD1a, and GalNAc-GD1a expressed on the motor axolemma are likely to be the epitopes for antibodies in AMAN. At the nodes or paranodes, deposition of antiganglioside antibodies initially cause reversible conduction block followed by axonal degeneration. Electrodiagnostic findings support this process. Disruption of glycolipids, which are important to maintain ion channel clustering at the nodes and paranode, may impair nerve conduction. Genetic polymorphisms of Campylobacter jejuni determine the expression of the gangliosides on the bacterial wall. In contrast, target molecules in AIDP have not yet been identified. Meta-analyses show efficacy of plasmapheresis and immunoglobulin therapy, but not corticosteroids, in hastening recovery.     

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an acute-onset, monophasic, immune-mediated polyneuropathy that often follows an antecedent infection. The diagnosis relies heavily on the clinical impression obtained from the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually provide evidence supportive of the diagnosis. The clinician must also be familiar with mimics and variants to promptly and efficiently reach an accurate diagnosis. Intravenous immunoglobulin and plasma exchange are efficacious treatments. Supportive care during and following hospitalization is also crucial.     

HIV-associated Guillain-Barré syndrome

Human immunodeficiency virus-associated Guillain-Barré syndrome (HIV-GBS) has been reported since 1985. Based on previous reports, this neuropathy typically occurs early in HIV infection, even at seroconversion, prior to developing acquired immunodeficiency syndrome (AIDS). Patients with GBS and CD4 counts of <50 have been proposed to have cytomegalovirus (CMV) infection and empiric gancyclovir is recommended. We reviewed medical records of 10 patients with HIV-GBS at five hospitals from 1986 to 1999. The mean CD4 count was 367/mm(3) (range 55-800). GBS was the first symptom of HIV infection in three patients. Four patients had AIDS with CD4 counts ranging from 55 to 190. CSF white blood cell (WBC) was 0 wbc/mm(3) in four patients, 2-10 wbc/mm(3) in three and 11-17 wbc/mm(3) in two. Three had recurrent weakness from 9 weeks to 4 years after the onset of symptoms, which persisted. HIV-GBS occurs in early and late stages of HIV infection, and may follow the onset of AIDS. No patients were seen with severe immunosuppression (CD4<50). A mild cerebrospinal fluid (CSF) pleocytosis in GBS suggests HIV infection, but is frequently absent. Compared to HIV-negative people, HIV-GBS may be associated with more frequent recurrent episodes or the development of CIDP.     

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.     

Sensory Guillain-Barré syndrome

OBJECTIVE: To report eight cases of sensory Guillain-Barré syndrome (GBS). BACKGROUND: The concept of sensory equivalent to ascending paralysis of GBS was raised in 1958, and the diagnostic criteria for a sensory loss and areflexia variant of GBS were proposed in 1981. However, clinical cases meeting these criteria have been relatively scarce. METHODS: During a 13-year period between 1986 and 1999, the authors collected eight cases of an acute sensory demyelinating neuropathy that met most of the proposed diagnostic criteria of a sensory variant of GBS. RESULTS: In all patients, sensory neuropathy was sudden at onset and peaked to maximal deficit within 4 weeks. In five (63%) cases, there was an antecedent viral illness. All patients had objective sensory loss and diminished or absent reflexes. None showed any muscle weakness. In all four patients in whom the spinal fluid was examined during the first 4 weeks, there was albuminocytologic dissociation. All of the patients had electrophysiologic evidence of demyelination in at least two nerves. Demyelination was demonstrated in motor nerve conduction in seven patients and in sensory nerve conduction in one, indicating that motor nerve conduction studies were the key for the diagnosis of demyelinating neuropathy. All patients had sensory nerve conduction abnormalities in at least one nerve. Three patients responded to immunotherapies. All had a favorable outcome, with a monophasic course of disease and no sign of relapse. CONCLUSION: The current study confirms the existence of sensory GBS.     

Guillain-Barré syndrome.

The concepts of Guillain-Barré syndrome have changed substantially over the last 10 years, and the last 2 years have been no exception. Guillain-Barré syndrome is now recognized as a heterogeneous disorder with many clinical manifestations. Most current investigations are centered on the hypothesis of molecular mimicry. The major challenge now is to identify the precise mechanisms of nerve fiber injury and to determine how to prevent immune injury.     

Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation

OBJECTIVE: To analyze long-term recovery and predictors of outcome in patients with Guillain-Barré syndrome (GBS) requiring mechanical ventilation. METHODS: The clinical and electrophysiologic data of 114 patients with GBS admitted to the intensive care unit between 1976 and 1996 (60 mechanically ventilated, 54 nonventilated) were reviewed. Functional disability and predictors of outcome were determined at 1 year and at maximal recovery using the Hughes scale. Good outcome was defined as ability to ambulate without assistance; poor outcome was defined as inability to ambulate independently. RESULTS: Mechanical ventilation was required in 81% of patients with a poor outcome. Mortality was 20% in patients ventilated for GBS. However, ventilated patients who survived did well, with 79% eventually regaining independent ambulation. Nineteen percent of patients improved at least one functional grade beyond 1 year. Univariate predictors of poor maximal recovery in ventilated GBS patients were increased age (p < 0.001)), upper limb paralysis (p = 0.004), duration of ventilation (p = 0.006), and delay of more than 2 days to transfer to a tertiary center (p < 0.001). However, only age (OR 1.99, p = 0.004) and delayed transfer (OR 19.8, p = 0.002) were independently predictive of poor outcome on multivariate analysis. CONCLUSION: Mechanically ventilated patients constitute the majority of GBS patients with a poor outcome, and mortality remains substantial in this subgroup (20%). Although recovery from severe GBS may be prolonged, most survivors regain independent ambulation.     

Guillain-Barré syndrome coexisting with pericarditis or nephrotic syndrome after influenza vaccination

A 68-year-old woman and a 72-year-old man presented with distal weakness of the limbs and numbness following an influenza vaccination within 2 weeks. Moreover, Guillain-Barré syndrome (GBS) was diagnosed in two patients. Pericarditis was diagnosed in the first patient who also had precordial chest pain with referral to trapezius ridge, and nephrotic syndrome, was observed in the second patient who had leg edema and proteinuria. The relationship among GBS, pericarditis and nephrotic syndrome after an influenza vaccination is discussed.     

Guillain-Barré syndrome and optic neuritis after Mycoplasma pneumoniae infection

We report the case of a 12-year-old girl who developed Guillain-Barré syndrome (GBS) and optic neuritis (ON) following Mycoplasma pneumoniae infection.Her symptoms, including bilateral vision impairment and tingling in her hands and right foot, were resolved after methylprednisolone pulse therapy. Serum anti-galactocerebroside (Gal-C) IgM antibodies were detected in our patient.This is the first report of a child with GBS and ON associated with M. pneumoniae infection.     

Guillain-Barré syndrome after brachial plexus trauma: case report

The Guilllain-Barré syndrome (GBS) is an acute predominantly demyelinating polyneuropathy. In many cases GBS is preceding by infection, immunization, surgery or trauma. Although there are a few reports of GBS after head trauma, there is no report of this syndrome after brachial plexus injury. We report on a 51 years-old man who presented GBS fifteen days after a brachial plexus trauma. The polineuropathy resolved completely in a few weeks. We believe that GBS was triggered by the trauma that evoked an immune mediated disorder producing inflammation and demyelination of the peripheral nerves.