vaccines were up to date , except for a booster dose of the combined diphtheria , tetanus , and inactivated poliovirus vaccine . his mother presented a common variable immune deficiency that was treated with weekly subcutaneous immunoglobulin . the symptoms began with a high fever around 39c , coughing , pain in the legs , and dysesthesia with a waddling gait . his sister had had a flu - like syndrome 1 week before . at first , the treatment was symptomatic . aggravations occurred 5 days later with the deterioration of the patient s general state , abdominal pain , food intolerance , and acute respiratory failure . on arrival at the pediatric emergency department , he presented tachycardia ( 150 beats / min ) , normal blood pressure , respiratory retraction , abdominal breathing , diffuse rhonchi , and severe hypoxemia ( oxygen saturation of 70% ) without hypercapnia . a brief neurological examination was unremarkable . he required noninvasive ventilation by high - flow oxygen therapy with up to 65% fio 
 2 . the next day , a chest x - ray control showed right basal consolidation . at that time , his parents reported a dribble on the way to the hospital with probable aspiration . on day 2 , a strikingly rapid neurological deterioration occurred , which consisted in agitation and in bilateral mydriasis but not clonism , and gave rise to suspicions of convulsion , which led to intubation , sedation , and mechanical ventilation . the neurological manifestations progressed to an unclassical bulbar and peripheral motor deficit , predominant on the upper limb with a brief central involvement . all those manifestations were noticed after respiratory stabilization occurred , and sedation was released on day 4 . he could not speak because of ventilation but could communicate with eye movements and blinking . dysfunctions in the cranial nerves became progressively evident and included facial diplegia , bilateral ptosis and mydriasis without ophthalmoplegia , severe swallowing disorders , and the absence of cough . it was associated with a brief hyperreflexia without a babinski reflex for 2 days and then with areflexia , leading to the possibility of the guillain - barr syndrome . dysautonomia was not clearly diagnosed , but the patient needed 4 vascular fillings in the first 3 days to be hemodynamically stabilized . the blood test showed inflammatory signs with increased c - reactive protein 116 mg / l ( n < 5 mg / l ) and procalcitonin 11 g / l ( n < 0.5 other microbiological tests ( lyme , botulism , respiratory viruses , bacteria and fungi , hiv , pneumocystis , and aspergillum ) and immune tests were normal . two examinations of cerebrospinal fluid performed on days 2 and 5 did nt reveal a cytoalbuminological dissociation ( white blood cells maximum 5/mm , normal glucose , normal protein and lactate levels , and sterile ) or oligoclonal bands . the analysis for antiganglioside and antigalactocerebroside antibodies detected nonspecific anti - gm4 on day 2 and were negative on day 26 . two cerebromedullar magnetic resonances on days 5 and 11 were normal , without any enhancements of the nerve roots . an ultrasonography was performed on day 21 , when the patient was still completely dependent on invasive ventilation . an electromyogram on day 8 ( figure 1 ) showed a normal lower limb and the decreased amplitude of motor responses on the upper limb , progressing into an axonal affection on the control electromyogram performed on day 18 ( figures 2 and 3 ) , confirming the diagnosis of an axonal motor polyneuropathy . electromyogram recordings of motor nerves on day 8 . decreased amplitude of motor responses on the upper limb . electromyogram recordings of motor nerves on day 18 . decreased amplitude of motor responses on the upper limb . the medical management of the patient consisted in administering a first course of intravenous immunoglobulin ( 1 g / kg / d for 2 consecutive days ) on days 5 and 6 . it was prescribed because of clinical severity , despite the lack of confirmation on the diagnosis . three weeks later , the authors decided to conduct a second course of immunoglobulin because the patient remained tetraparetic and still depended on assisted ventilation . when the patient was admitted , he was suspected of being immunodeficient and was therefore treated with a large probabilistic association of medication , including acyclovir , clarithromycin , ceftriaxone , cotrimoxazole , and oseltamivir . of all the medication , clarithromycin was the only drug that was administered for 21 days . clavulanate was associated with it for 8 days in order to treat ventilator - assisted pneumonia . however , the oxygen requirement rapidly decreased , but muscle weakness and swallowing troubles compromised autonomous breathing for a long time . since the neurological prognosis was guarded , a tracheostomy was performed on day 29 , improving comfort . enteral feeding for 18 weeks ( first with a gastric tube and then through gastrostomy from day 55 ) and salt supplementation , because of intense salt loss when swallowing , were required . once life - threatening complications were excluded , the patient started intensive functional and psychotherapeutic rehabilitation . the patient s progress was slowly favorable , and he left the hospital after 11 weeks . an examination after 20 weeks only showed a proximal weakness and decreased upper limb deep tendon reflexes . the tracheostomy and the gastrostomy could be removed after 5 months without any complications . during the last examination , 1 year after the patient s admission to the hospital , the authors still noticed discrete hypotonia in the upper limb with fine motor skill difficulties , fatigability , and intermittent hand tremors . the authors present the case of an atypical and severe axonal guillain - barr syndrome , associated with a m pneumonia lung infection , in a 7-year - old boy . the authors initially focused on respiratory distress , due to the fact that our patient first presented febrile acute respiratory failure linked to an interstitial pneumonia , in a context of pain and probable weakness of the lower limb . lower limb weakness seemed to be the first sign , but it was associated with pain and dysesthesia , which mimicked flu - like diffuse myalgia . neurological disorders were only noticed when vital respiratory functions had been managed through mechanical ventilation and through the release of sedation . the respiratory emergency was the main focus when the patient was admitted with severe hypoxemia . it is well known that in emergency departments , where clinical presentations can be elusive at first , misdiagnoses or delayed diagnoses are not uncommon . the authors did nt observe the typical delay that usually occurs between the infectious trigger and the acute polyneuropathy . the authors recall the fact that m pneumonia can be the trigger for large peripheral and central neurological manifestations , through several sorts of pathomechanisms , making it more difficult to identify the correct diagnosis . second , neurological symptoms were misleading because of the association between central and peripheral involvement disorders : involvement of cranial nerves , tetraparesia that was predominant on the upper limb , and deep tendon reflexes first noted as normal , then brisk , and finally depressed . however , there was nt a disturbance of consciousness , nor was there ophthalmoplegia , or ataxia . the 2 lumbar punctures did nt serve as support , as they did not document any albuminological dissociations . the examination of antiganglioside and antigalactocerebroside antibodies did nt help either and were both negative . the repeated use of the electromyogram was the only tool that led to the certain diagnosis of an axonal , bulbar , and predominant on the upper limb guillain - barr syndrome . this case report highlights the fact that the guillain - barr syndrome is a large nosologic framework that encompasses several clinical variants and overlaps with bickerstaff s brain stem encephalitis . this forms a continuous spectrum in which the diagnosis of especially atypical cases can be particularly difficult to establish . however , establishing an early diagnosis and thus initiating a specific immunotherapy treatment is a real challenge . in severe cases , the cochrane database recommends intravenous immunoglobulin . in this case , the first course of immunoglobulin was only administrated on days 5 and 6 , although it started before the electromyogram confirmation , which points to the difficulties met to find the diagnosis . the intravenous immunoglobulin treatment did not contribute to make significant improvements . because of the severity of such atypical forms , and due to the guarded neurological prognosis in such cases , the authors decided to administer a second course 3 weeks later . this strategy is rare but recommended for severe guillain - barr syndrome cases . determining the nosologic framework was also important since the question of adjuvant treatments  such as intravenous corticoids  in the event of bickerstaff s encephalitis had been discussed . finally , reanimation supporting care was essential for our patient who needed 48 days of invasive ventilation assistance and was therefore in the most severe range of ventilation duration . of the patients with guillain - barr syndrome , 17% to 30% require mechanical ventilation with an increased risk of death . nearly 5% of patients with guillain - barr syndrome die from medical complications such as sepsis , pulmonary emboli , or unexplained cardiac arrest . the mean duration of ventilation ranges between 15 and 43 days , suggesting that a proportion of patients can receive a tracheostomy for better comfort and airway safety . 
  usually , poor prognostic factors are related to age , clinical stages , and electrophysiological subtypes ( axonal form ) . this confirms the severity of our case and the urgent need to provide a correct diagnosis and immunotherapy to fight against a poor outcome . this case report highlights the fact that respiratory distress can be the main clinical symptom in children , thus delaying the correct diagnosis of guillain - barr syndrome , even more so when rare and unusual neurological manifestations are combined , although they have already been reported separately within the paediatric population . this case also highlights the importance of recognizing the constellation of guillain - barr symptoms across a spectrum , especially in children , in order to ensure the rapid and efficient medical management of patients , particularly in severe forms .
guillain - barr syndrome is a rare acute polyradiculoneuropathy . 
 several variants and unusual presentations have been described , particularly in pediatrics . in most cases , making an early diagnosis is challenging due to the treatments that consist in the rapid administration of intravenous immunoglobulin or plasma exchange . 
 the authors present the case of a 7-year - old boy with an atypical and severe axonal guillain - barr syndrome , associated with mycoplasma pneumonia . 
 when he was admitted , febrile respiratory failure was the main focus , and then he presented signs of acute polyneuropathy with cranial nerve palsy and brief hyperreflexia . 
 mechanical ventilation was required for 48 days as well as 2 cycles of intravenous immunoglobulin . 
 the authors describe all the medical challenges that the authors encountered . 
 this case highlights the fact that respiratory distress can be the main clinical symptom in children . 
 this delays the establishment of a correct diagnosis , even more so when neurological manifestations are abundant and unusual .