Hyponatremia in Guillain-Barre Syndrome: A Review of Its Pathophysiology and Management.

Guillain-Barre syndrome (GBS) is the commonest cause of acute polyradiculoneuropathy that requires hospitalization. Many of these patients experience systemic and disease-related complications during its course. Notable among them is hyponatremia. Though recognized for decades, the precise incidence, prevalence, and mechanism of hyponatremia in GBS are not well known. Hyponatremia in GBS patients is associated with more severe in-hospital disease course, prolonged hospitalization, higher mortality, increased costs, and a greater number of other complications in the hospital and worse functional status at 6 months and at 1 year. Though there are several reports of low sodium associated with GBS, many have not included the exact temporal relationship of sodium or its serial values during GBS thereby underestimating the exact incidence, prevalence, and magnitude of the problem. Early detection, close monitoring, and better understanding of the pathophysiology of hyponatremia have therapeutic implications. We review the complexities of the relationship between hyponatremia and GBS with regard to its pathophysiology and treatment.

Tropical ataxic neuropathy - A century old enigma.

Tropical ataxic neuropathy, which is prevalent in the tropics causes significant disability as well as increased mortality and remains an enigmatic disease with no effective treatment or cure, even a century after its identification. The syndrome, first described in Jamaica in 1897 and christened as tropical ataxic neuropathy in 1959, is a constellation of bilateral optic atrophy, bilateral sensory neural deafness, predominant posterior column involvement and pyramidal tract myelopathy, with ataxic polyneuropathy. The exact etiopathogenesis remains unresolved, and several factors have been proposed including malnutrition, vitamin B deficiencies, malabsorption, poor protein consumption, chronic cyanide, and nitrile toxicity, with a strong geospatial endemic prevalence in areas of cassava cultivation. In this review, we summarize the history, epidemiology, clinical features, and controversies regarding the pathogenesis and differential diagnosis of the disease and identify the potential areas for further research concerning this debilitating disorder that is common in the tropics. Its multifactorial etiopathogenesis provides potential opportunities for research and international collaboration to identify novel avenues for treatment.

Electrophysiological observations in critically ill Guillain-Barre syndrome.

BACKGROUND: Respiratory muscle paralysis is a serious complication of Guillain-Barre syndrome (GBS). Factors that govern duration and recovery from respiratory paralysis are unclear. AIM: To correlate electrophysiological parameters in critically ill GBS with duration of mechanical ventilation and outcome at discharge. MATERIALS AND METHODS: Data of a large cohort (n=93; M:F 59:34; mean age: 33.51+21.4 years) of critically-ill patients with GBS seen over one decade was retrospectively analyzed. RESULTS: The duration of mechanical ventilation was <15 days (n = 38), 16-30 days (n = 24), and >30 days (n = 31). Majority of the patients had a demyelinating electrophysiology. Reduced amplitude or absent motor potentials correlated with requirement for longer duration of ventilation. Inexcitable sensory nerves were more common in patients who could be weaned off from the ventilator within 15 days. There was no relation between the conduction blocks in motor nerves and the duration of ventilation. Low amplitude of median nerve correlated with a poor outcome at hospital discharge as assessed by Hughes disability scale. CONCLUSION: Distinct patterns of electrophysiological abnormalities are noted in patients and they correlate with the duration of mechanical ventilation. Future studies to unravel the underlying pathophysiological processes that govern the patterns of progression and recovery in the critically ill patients with GBS will pave way for the development of better and more potent therapies that will hasten recovery, when combined with the prevalent treatment modalities including plasmapheresis and intravenous immunoglobulin.

A comparison of immunomodulation therapies in mechanically ventilated patients with Guillain Barré syndrome.

A comparison of the effectiveness of immunomodulatory therapies in patients with Guillain Barré syndrome (GBS) who require mechanical ventilation (MV) is important for patient treatment and cost. We aimed to compare the effectiveness of three modes of intervention on the outcome of patients with GBS receiving MV: intravenous immunoglobulin (IVIgG); small volume plasmapheresis (SVP) and large volume plasmapheresis (LVP). Patients with GBS satisfying National Institute of Neurological and Communicative Disorders and Stroke 1990 criteria and requiring MV between 1997 between 2007 were analyzed. The primary outcome parameters evaluated were mortality, duration of MV, hospital stay and Hughes scale at discharge from hospital. Of the 173 (Male: Female, 118:55) patients who required MV during the study, 106 patients received single modality treatment (IVIgG 31, LVP 45, SVP 30) based on availability, affordability and feasibility. Patients receiving IVIgG had a higher incidence of severe weakness and bulbar involvement. The mean duration of MV (p=0.61), total hospital stay (p=0.44) and Hughes scale at discharge (p=0.31) did not differ among the three groups. Complications were similar in the three treatment groups except for hypoalbuminemia and anemia, which were more common in patients in the LVP group. In conclusion, the outcome of patients treated with these three immunomodulatory treatment modalities did not vary. The beneficial effects of SVP in our study warrant further randomized control trials especially in resource-constrained settings.

An unusual case of unilateral limb hypertrophy: Lipoma of sacral roots.

We report an unusual case of unilateral limb pseudo hypertrophy in a 21-year-old lady who developed progressive enlargement of the right calf followed by thigh in association with chronic leg pain. Magnetic resonance imaging (MRI) of the affected limb confirmed enlargement of various muscles. Electromyography revealed neurogenic features consistent with S1 radiculopathy. MRI of the lumbosacral spine showed tethered cord with a lipoma infiltrating multiple sacral roots. Our case illustrates that muscular pseudo hypertrophy may follow chronic denervation as a consequence of spinal neural compressive disease. The various mechanisms postulated for this distinct condition are outlined.

Prognosis of patients with Guillain-Barré syndrome requiring mechanical ventilation.

INTRODUCTION: Severe Guillain-Barré syndrome (GBS) is associated with significant morbidity and also mortality. Identification of modifiable risk factors may help in reducing the morbidity and mortality. OBJECTIVE: To study the prognostic factors in a selected cohort of mechanically ventilated GBS patients. MATERIALS AND METHODS: Case records of GBS patients requiring mechanical ventilation admitted between 1997 and 2007 were analyzed. All patients satisfied the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for GBS. Primary outcome parameters included mortality and GBS disability (Hughes) scale score at discharge. RESULTS: During the study period, 173 (118 men and 55 women; mean age of 33.5 ± 21 years) GBS patients were mechanically ventilated. A history of antecedent events was present in 83 (48%) patients. In addition to motor weakness, In all facial palsy was present in 106 (61%), bulbar palsy in 91 (53%), sensory involvement in 74 (43%), and symptomatic autonomic dysfunction in 27 (16%). The overall mortality was 10.4%. On univariate analysis the risk factors for mortality included elderly age (P = 0.014), autonomic dysfunction (P = 0.002), pulmonary complications (P = 0.011), hypokalemia (P = 0.011), and bleeding (P = 0.026). All these factors were significant in multivariate analysis except for bleeding from any site and hypokalemia. In univariate analysis factors associated with Hughes scale score ≤ 3 at discharge included younger age (P = 0.02), presence of bulbar symptoms (P = 0.03) and less severe weakness at admission (P = 0.02), slower evolution of disease over more than 3 days (P = 0.01), electrodiagnostic evidence of demyelinating neuropathy (P = 0.00), and absence of sepsis (P = 0.01), hyperkalemia (P = 0.0001), and anemia (P = 0.02). In multivariate analysis age was the only significant factor. CONCLUSIONS: Early identification of modifiable risk factors, such as pulmonary involvement, autonomic dysfunction, hypokalemia, sepsis, bleeding, and nutritional complications, may reduce the mortality and morbidity associated with GBS.

Sleep in Wilson's disease: Questionnaire based study.

OBJECTIVE: We proposed to detect sleep abnormalities in Wilson's disease, (WD) using sleep questionnaires. MATERIALS AND METHODS: Twenty-five patients (M:F = 18:7; age: 24.4 ± 9.2 years) with WD and 24 controls (all males; age: 33.1 ± 9.7 years) were recruited. They underwent phenotypic/magnetic resonance imaging (MRI) evaluation followed by administration of Pittsburg Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS) questionnaires. RESULTS: The mean age at presentation and diagnosis was 24.4 ± 9.2 and 17.6 ± 7.5 years, respectively. The duration of illness at diagnosis was 14 ± 21.9 months. On PSQI, 15 patients with WD had abnormal PSQI scores of >5 as compared to 6 patients among the controls. The mean PSQI score was significantly more (P = 0.03) in patients compared to the controls. The PSQI worst scores were noted only in WD. Evaluation with ESS showed that three patients with WD scored >10/24, while two among the controls qualified for excessive daytime sleepiness. Overall, assessment by sleep questionnaires detected abnormality in 16 patients with WD as compared to 8 controls (P = 0.004). Subgroup analysis revealed that patients whose duration of illness was >8 years and who were on decoppering treatment had significantly lesser excessive daytime somnolence. CONCLUSIONS: Sleep disturbances were observed more often in WD than in controls. Better designed studies will provide a better understanding.

Mortality in mechanically ventilated patients of Guillain Barré Syndrome.

BACKGROUND: The mortality of patients with Guillain Barré syndrome (GBS) has varied widely with rates between 1-18%. Death results from pneumonia, sepsis, adult respiratory distress syndrome (ARDS) and less frequently due to autonomic dysfunction or pulmonary embolism. There are only few studies which have used a large sample and have in detail analyzed the circumstances relating to death and the prognostic factors for the same in a cohort, including only mechanically ventilated patients. OBJECTIVE: The objective of our study was to analyze the circumstances and factors related to mortality in mechanically ventilated patients of GBS. MATERIALS AND METHODS: Case records of patients of GBS, satisfying National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria, and requiring mechanical ventilation from 1984 to 2007, were analyzed. RESULTS: A total of 273 GBS patients were managed with ventilatory support (190 men and 83 women) during the period. Besides symmetrical paralysis in all patients, bulbar palsy was present in 186 (68.1%), sensory involvement in 88 (32.2%) and symptomatic autonomic dysfunction in 72 (26.4%) patients. The mortality was 12.1%. The factors determining mortality were elderly age group (P=0.03), autonomic dysfunction (P=0.03), pulmonary complications (P=0.001), hypokalemia (P=0.001) and bleeding (P=0.001) from any site. Logistic regression analysis showed the risk of mortality was 4.69 times more when pneumonia was present, 2.44 times more when hypokalemia was present, and 3.14 times more when dysautonomia was present. The odds ratio for age was 0.97 indicating that a higher age was associated with a higher risk of mortality. CONCLUSIONS: Ventilator associated pulmonary complications, bleeding and hypokalemia especially in elderly patients require optimal surveillance and aggressive therapy at the earliest for reducing the mortality in this group of GBS patients.

Sleep in Wilson's disease: a polysomnography-based study.

Wilson's disease (WD) has neuro-anatomical, pathophysiological and neurochemical basis for sleep disturbances. The aim of the study was objective evaluation of the frequency and nature of sleep abnormalities using polysomnography (PSG) in patients with WD. The study included 25 subjects with WD (males, 18; age , 24.4 ± 9.25 years) and 25 healthy controls (all males; age, 33.1 ± 9.7 years). After phenotypic assessment and magnetic resonance imaging (MRI), sleep-related questionnaires were administered, and PSG was performed. Patients had significantly reduced total sleep-time (P=.001), sleep-efficiency (P=.001), percentage of deep sleep (P=.01), and REM-sleep (P=.04) with prolonged sleep-onset latency (P=.05) and latency to stage 2 (P=.02). Subgroup analyses of patients based on demographic and clinical parameters were done. Men had significantly more bradycardia both during awake (P=.002) and sleep (P=.03) states. Younger patients (<20 years) had frequent tachycardia (P=.01), higher Periodic Limb Movement (PLM) Index (P=.01) and lesser REM% sleep (P=.05). Patients on de-coppering therapy had prolonged REM-sleep-onset latency (P=.03) and mixed apnea events (P=.04). The isolated limb movements were more in the severe form of disease (P=.05) and in patients taking anticonvulsants (P=.03). This study, the first of its kind in literature, revealed significant sleep disturbances in patients with Wilson's disease.