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Letter to the Editor
Improvement in Lasegue’s Sign: An Early Predictor of Recovery in Childhood Guillain-Barre Syndrome Sir, Lasegue’s sign (painful limitation of straight-leg raising) is a feature of radiculopathy and is commonly reported in childhood Guillain-Barre Syndrome (GBS).1 Improvement in limitation of straight-leg raising (SLR) has been used to monitor clinical recovery in lumbar disc herniation,2,3 however, the same has not been evaluated in respect to GBS. We present 3 patients of GBS, in whom improvement in SLR heralded the disease recovery before any other quantitatively demonstrable sign. Nine patients of childhood GBS were managed in Pediatric Intensive Care Unit of a tertiary care center over 3 years period (Sept’2000 to June’03). Three patients (age, 4 -6 years) had Lasegue’s sign at admission. All the three had progressive ascending symmetrical hypotonic hyporeflexic motor weakness, which developed over 3-7 days. Motor power varied from 1/5 to 3/5. All had intercostal muscle weakness, neck rigidity, leg pains, positive Kernig’s sign and 1 had backache. Angles of SLR in third patient were 0º and 30º in right and left legs respectively. He also had autonomic instability in the form of varying heart rate (100-124/min) and blood pressure (90/60-110/70 mmHg) during first 36 hours. EKG was normal. None had had dog bite, immunization or viral illness in recent past, or cranial nerve involvement. Routine investigations were normal in 2 patients. One had hypokalemia (serum potassium 3.2 mEq/L) and compensated respiratory acidosis (pH 7.42, PaCO2 47.7 torr, HCO3- 30.6 mEq/L); limb and intercostal weakness persisted despite correction of hypokalemia. Cerebrospinal fluid examination was performed in 2 patients (on day 6 and day 7) and was normal. Neurophysiologic studies could be obtained in 1 on day 7 of illness, which revealed normal nerve conduction velocity (45.2 - 46.9 m/sec), decreased amplitude (left ulnar nerve, 0.12 mV; left posterior tibial nerve, 0.73 mV) and increased latency (left ulnar nerve, 5.04 msec left posterior tibial nerve, 8.04 msec) in motor nerves. Stool cultures for polioviruses were negative. Patients were diagnosed as Guillain-Barre Syndrome based on Asbury’s modified clinical criteria4 with grade 4 disability.5 Patients underwent double-volume exchange transfusion 6 (with 120 ml/Kg blood) on day 2 for respiratory involvement and progressive motor weakness, as neither plasmapheresis nor intravenous immunoglobulins was available to us. After therapy, Lasegue’s sign had improved in 2 patients on the very next day, while in one on day 3. In the third patient, angles of SLR improved to 30º (right) and 45º (left) on day 3, which improved further to 75º (right) and 60º (left) by Indian Journal of Pediatrics, Volume 73—March, 2006
day 5. Improvement in Lasegue’s sign was followed by consistent and continued motor recovery in all 3 patients. Improvement in motor power by at least 1 grade in at least 1 limb was demonstrated for the first time on day 5, 4 and 5 of exchange transfusion respectively. They were discharged after 7-12 days of hospitalization. At 4-6 months follow-up, all patients made complete motor recovery. Radiculopathy is an early and prominent feature of GBS7; and thus Lasegue’s sign, a neurodynamic test for radiculopathy, is reported to present in majority (83%) of childhood cases 1. Though intensity of radicular pain syndromes was not found to correlate with motor disability and may precede or follow the motor weakness,8 improvement in Lasegue’s sign, if present, may occur along with recovery in radiculopathy. Among signs of radiculopathy, Lasegue’s sign is the only clinical tool that can be quantified objectively. Improvement in SLR was observed in initial two patients, followed by motor recovery 3-4 days after. Having realized the potential prognostic value of improvement in SLR, its quantitative monitoring was performed in the third one. In this patient, SLR improved by 15 0-300 within 24 hours of therapy, while motor power improved only after 5 days. Till date, only motor signs, viz. halt in progression of motor weakness, number of days required to improve two disability grades, need and duration of ventilatory support and degree of maximum weakness on day 10 have been used to prognosticate outcome and to compare efficacy of various therapeutic modalities.9,10 These motor signs may not be helpful as early prognostic predictors, for motor improvement is usually slow. Improvement in SLR as a measure of recovery in radiculopathy may be an alternative which is already being used as an early prognostic indicator in patients with nerve root involvement in lumbar disc herniation. 2,3 Patients in the present study demonstrated significantly earlier response in SLR as compared to motor power. Quantitative monitoring of improvement in Lasegue’s sign, if present, may be a good clinical tool for early prognostication and for comparing short-term effects of various therapeutic modalities. A study on larger number of patients will be required to document the prognostic value of this sign in patients with GBS. Arun K. Baranwal1, Ravi N. Mandal and Rupa Singh Department of Pediatrics, B.P. Koirala Institute of Health Sciences, Dharan, Nepal and Government Medical College and Hospital, Chandigarh, India E-mail:
[email protected] 249
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Letter to the Editor REFERENCES 1. Nguyen DK, Agenarioti-Belanger S, Vanasse M. Pain and the Guillain-Barre syndrome in children under 6 years old. J Pediatr 1999; 134: 773-776. 2. Graham GE. Intraoperative straight-leg raising during laminectomy and disk excision for sciatica. Clin Orthop 1981; 154: 343-434. 3. Vroomen PC, de Krom MC, Knottnerus JA. Predicting the outcome of sciatica at short-term follow-up. Br J Gen Pract 2002; 52: 119-123. 4. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for the diagnosis of Guillain-Barre Syndrome. Ann Neurol 1990; 27(Suppl): S21. 5. Guillain-Barre Syndrome Study Group. Plasmapheresis and acute Guillain-Barre Syndrome. Neurology 1985; 35: 1096.
6. Singh S, Singhi S. Recovery from respiratory paralysis caused by Guillain-Barre Syndrome in an infant after repeated exchange transfusions. Clin Pediatr 1989; 28: 480-481. 7. Lebrun C, Ghetau G, Candito M, Chatel M. Acute polyradiculoneuritis and dysautonomia: contribution of assaying catecholamines and their methoxylated metabolites. Rev Neurol (Paris) 2001; 157: 304-308. 8. Moulin DE, Hagen N, Feasby TE, Amireh R, Hahn A. Pain in Guillain-Barre syndrome. Neurology 1997; 48: 328-331. 9. Shanbag P, Amirtharaj C, Pathak A. Intravenous immunoglobulins in severe Guillain-Barre Syndrome in childhood. Indian J Pediatr 2003; 70: 541-543. 10. Ortiz Corredor F, Mieth Alviar KW. Prognostic factors for walking in childhood Guillain-Barre Syndrome. Rev Neurol 2003; 36 : 1113-1120.
Persistent Pulmonary Hypertension among Neonates with Sepsis Sir, Persistent Pulmonary Hypertension of the Newborn (PPHN) is caused by a sustained elevation of pulmonary vascular resistance (PVR) at birth. A variety of events may interfere with this process of transition. Parenchymal lung disease, meconium aspiration, intrauterine/perinatal hypoxia, group B streptococcal pneumonia or sepsis, abnormal pulmonary development, severe hyaline membrane disease and cardiac diseases are known to be associated with PPHN. Sepsis is the commonest among these clinical problems. Thus in some cases of sepsis, rapid breathing may also be due to pulmonary hypertension and not due to pneumonia alone. Here is a report which records the experience of managing six newborns with sepsis and pulmonary hypertension. The six newborns were between 34 weeks to 38 weeks gestational age and had birth weight between 1.75 kg to 2.8 kg, admitted to neonatal intensive care unit for respiratory distress since birth. Cord blood gases revealed pH between 6.8 and 7.21 and base deficit between - 8.1 and 25.8. Chest X-rays were normal in all the subjects. Complete blood counts (CBC) done on day one of life suggested sepsis in five babies.1 I/T ratio was increased in one case (0.21); in the remaining cases the ratio ranged between 0.02 to 0.14. The blood cultures grew pseudomonas in 2, acinetobacter in 1, mixed growth of Klebsiella and coagulase negative staphylococci in 1, and coagulase negative staphylococci in 1 case. Portable echocardiographic findings included dilatation of leftsided chambers in 1, right-sided chambers in 1, all the chambers in 2 and hypocontractile heart in 1 case. Pulmonary hypertension was diagnosed from high initial pulmonary artery pressure (PAP), more than 2/3 of normal mean arterial pressure (MAP) for given birth weight in 3 cases (49,50,66)mm of Hg and from 250
substantial increase in repeat echocardiogram in 3 cases (33 to 73, 28 to 50, 37 to 51) 2 mm of Hg. These babies received oxygen by hood, inotropes (adrenaline 0.1 mcg/ kg/min + dobutamine 10 mcg/kg/min), amrinone (0.75 mg/kg bolus, followed by 10 mcg/kg/min infusion) and antibiotics in view of respiratory distress with evidence of perinatal asphyxia and sepsis. Disappearance of tachypnea and chest in drawings was taken as an indicator of decrease in PPHN, as follow-up of echocardiography was not feasible. Four babies survived. Respiratory distress is a common neonatal problem. In presence of markers of infection like peripheral blood counts or positive blood culture, pneumonia is commonly diagnosed. Chest X-rays in neonatal pneumonia are often normal. However, in some cases of sepsis, respiratory distress may be due to PPHN. Severe infection can produce factors that cause constriction of lung vessels. Resultant hypoxia may cause further pulmonary vasoconstriction as well as decreased myocardial contractility. Early intervention may lead to favorable outcome. Specific interventions include administering oxygen; a natural pulmonary vasodilator and inotropes like dopamine, dobutamine, epinephrine, isoproterenol to decrease pulmonary blood pressure; maintain systemic blood pressure, reverse the right to left shunting and treat the underlying cause.3 Magnesium sulphate infusion was found to be effective in PPHN in nine newborn infants with severe pulmonary hypertension, seven of which survived, suggesting a beneficial effect. 4 Magnesium sulphate infusion has been used in nonventilated babies with PPHN in lower dose of 50 mg/kg/hr without encountering respiratory depression.5 Milrinone and amrinone are frequently used inodilators (inotrope/ vasodilator) known to have favorable effect by lowering pulmonary pressure and enhancing inotropic effect. 6 Indian Journal of Pediatrics, Volume 73—March, 2006
