A 12-year-old boy with previous history of upper respiratory tract infection was referred to outpatient clinic of Mofid Children Hospital because of legs pain, walking instability, and lower extremity weakness. His problems were started since 5 days ago. The past medical history revealed that he was a third child of the family (III/III). There was a familiar relationship between his parents (cousin relationship). Furthermore, his 22-year-old sister had a history of lupus. His mother had also the history of three abortions. On physical examinations, he had decreased in deep tendon reflexes (DTR). His first biochemical tests results were as following: white blood count = 3,650/mm3 (neutrophil: 44% and lymphocyte: 56%); hemoglobin = 13 mg/dL; and platelet = 259,000/mm3, erythrocyte sedimentation rate = 29 mm/hrs; C-reactive protein = negative. Laboratory tests for antinuclear antibodies (ANA) and anti-ds-DNA were provided to rule out lupus. The results of laboratory tests were positive for both ANA (1:2,560) and anti-ds-DNA antibodies. Cerebellar examination revealed abnormal finger-to-nose and tandem gait tests. Analysis of cerebrospinal fluid, brain magnetic resonance imaging (MRI), chest X-ray and abdominal-pelvic ultrasonography results were within normal limits. His electromyography (EMG) and nerve conductive velocity (NCV) study showed absent in compound sensory nerve action potentials. Therefore, he was diagnosed as having GBS according to Asbury criteria and admitted and received intravenous immunoglobulin (IVIG). However, there was no appropriate response by treatment and he developed severe headaches which were associated with disrupted sleep. Due to the presence of leukopenia during his admission, bone marrow aspiration was provided which was normal.
Considering polyneuropathy involving all limbs diagnosis was in favor of acute inflammatory demyelinating polyneuropathy. Cardiology consult was performed that showed mild mitral regurgitation and tricuspid regurgitation. Additional biochemical test analysis, as like as creatine phosphokinase; lactate dehydrogenase; thyroid function tests; Immunoglobulin levels, anticardiolipins antibodies; anti-smith antibodies; beta2 glycoproteins antibodies; and lupus anti-coagulant all were negative or within normal limits. Serological test results for brucellosis, mononucleosis, tuberculosis, bacterial and fungal and his throat culture were also negative. Random and 24 hrs urinalysis test results were normal. The patient general condition was relatively improved during the hospitalization. Gait disorder was improved and the patient was discharged to be followed up.
Two weeks later, the patient was again admitted to our hospital with the complaint of legs pain, loss of balance, walking instability, lower extremity weakness, and anorexia. He had also ataxic gait. Cerebellar test disorders and DTR in lower limbs were observed as abnormal examinations. Distal force in upper and lower limbs was decreased. The patient was then admitted again in neurology ward. Lumbar puncture was performed again and auto-antibodies titration for GBS were checked which were in normal ranges. The serum level of vitamin B12 and vitamin E was measured to rule out the neuropathy that was normal. Total spinal MRI was normal. EMG and NCV tests were performed which reported chronic mild sensory motor demyelination in upper and lower limbs. The other biochemical and CBC tests results were performed again and showed normal results. The patient's general condition was gradually improved after supportive care and then be discharged.
However, he admitted again because of decreased force in upper and lower limbs (2/5), and absence of DTR in lower limbs due to the possibility of chronic inflammatory demyelinating polyneuropathy (CIDP). EMG and NCV tests showed chronic mild sensory motor demyelination in upper and lower limbs. Polyneuropathy involving all limbs suggestion was provided for CIDP. After CIDP diagnosis, the patient treated with the pulse of methylprednisolone for 5 days. He received oral prednisolone for 3 months and then gradually tapered. One month after stopping prednisone therapy, the patient developed severe upper and lower limbs weakness, walking instability, and gait disorders. The patient had decreased DTR in lower limbs under physical examinations. Finger-to-nose problems and tandem gait disorders were observed under cerebellar tests. Laboratory tests were performed again due to the possibility of lupus. The test results were as follow: anti GM IgM+, anti-CD10 IgG+, ANA+ (1:2,560), anti-ds-DNA+, and beta2 glycoprotein IgM+. Proteinuria and hematuria were found under urinalysis. Leukopenia was found under several CBC tests analysis accompanied with low levels of complements. Eventually, the boy was transferred to division of Pediatric Rheumatology and treated with the pulse of methylprednisolone, hydroxychloroquine, and pulse of cyclophosphamide. His neurological and physical symptoms improved and had complete neurological recovery several months after his initial presentation.