Clinical course of an acute motor axonal neuropathy

patient with some prognostic factors treated with low

dose intravenous immunoglobulin
Yeremias Setyawan1, Sitti Yanhil1, Denny Pambudi2, Arthur H.P. Mawuntu3, Corry N. Mahama3, Barry I. Kambey4, Edward Nangoy5, Herlyani Khosama3
1) Student, Medical Doctor Program Faculty of Medicine Sam Ratulangi University/R.D. Kandou Hospital; 2) Resident, Neurology Department Faculty of Medicine Sam Ratulangi
University/R.D. Kandou Hospital; 3) Lecturer, Neurology Department Faculty of Medicine Sam Ratulangi University/R.D. Kandou Hospital; 4) Lecturer, Anesthesia and Intensive
Care Department Faculty of Medicine Sam Ratulangi University/R.D. Kandou Hospital; 5) Head of Pharmacy and Therapeutics Committee R.D. Kandou Hospital

Guillain-Barr syndrome (GBS) is a post-infectious immune-mediated peripheral neuropathy characterized by rapidly progressive
Background

weakness and sensory loss, usually followed by slow clinical recovery. The age distribution seems to be bimodal (ages 15-35 years
old and age 50-75 years old). GBS is heterogeneous in the severity of neurological deficits and prognosis. Some patients may
develop respiratory paralysis and remain bedridden or wheelchair bound while others may only have mild limb paresis that
recovers spontaneously within weeks. The advanced understanding of the diseases have led to a better outcome of the disease. The
effective treatments for GBS are plasma exchange (PE) or intravenous immunoglobulin (IVIg). Nevertheless, there are still about
20% patients remain in long term disability or died due to the complications of GBS. Old age, type of GBS, the severity of the
disease, and mode of treatment are some factors that influence the outcome.
A 63 years old male was admitted to the R.D. Kandou Hospital Emergency Room with a chief
Casae Reort

complaint of limb and body weakness for 5 days before admission. The weakness was progressive
and started from both feet and accompanied by a tingling sensation. On admission, the patient
also complained of breathing difficulty. There was no history of prior diarrhea but the patient got
flu 2 weeks ago that resolved spontaneously. Blood pressure was 110/60 mmHg, pulse 56 bpm,
respiration 24/minute, body temperature 36.7OC. There were no wheezing or crackles were
found on chest auscultation. On neurological examination, the swallowing function was
compromised, the muscle strengths were 1/2/2/2|2/2/2/1 on upper extremities and
1/2/2/2|2/2/2/1 on lower extremities (MRC scale). Muscle tone was decreased and all
physiological reflexes were absent. Counting test was 10 of 20. Mild leukocytosis (10.200/ul) was
found on initial hematological examination while the serum electrolytes, liver function test, and
kidney function test came back normal. Blood gas analysis showed respiratory alkalosis. The
patient was admitted to the ICU. Nerve conduction study (NCS) performed on the 2-nd day
showed decreased amplitude of right motoric median and ulnar nerves, and bilateral motoric Picture 1. Intial NCS.
peroneal and tibial nerves with normal nerve conduction velocities while the sensory nerves
were normal. Those findings supported the diagnosis of GBS acute motor axonal neuropathy
(AMAN) type. The Erasmus Guillain-Barre Respiratory Insufficiency Score (EGRIS) for the first
week was 78% (95%CI 60-89%). Based on those data, the medical team requested for IVIg
treatment and was approved by the Hospital Pharmacy and Therapeutics Committee on the 3-rd
day but only be given for three consecutive days using standard dose 0.4mg/kg BW.
One day after the last IVIg dose, the patient complained no breathing difficulty and the tingling
sensation was improved but still felt weak. The muscle strengths improved to 3/4/4/4|4/4/4/2
on upper extremities and 2/2/2/3|3/2/2/2 on lower extremities (MRC scale) and weak
physiological reflexes were detected. The patient was transferred to Neurology Intermediate Care
Ward on the 8-th day. The follow-up NCS on the 12-th day showed improvement compared to the
previous examination. The patient was discharged on the 15-th day. On one week follow up, the
dysphagia was absent, the muscle strengths improved to 3/4/4/4|4/4/4/2 on upper extremities
and 2/3/3/3|3/3/2/2 on lower extremities. Picture 2. Follow up NCS.

This case delineates a successful IVIg treatment in term of avoiding respiratory failure in severe GBS patient with some poor prognostic factors.
Discussion

However, the muscle strengths showed a slower recovery improvement causing a walking difficulty in this patient.
Intravenous immunoglobulin is an expensive treatment and not always readily available. So, in our case, the patient only received three
consecutive doses instead of five. This may be contributed to the partial recovery of the patient. However, not all patient will recover enough
after a standard IVIg treatment. Several clinical factors are associated with the outcome. The poor prognostic factors found in our patient were
the old age and the involvement of the bulbar muscles. A low increase of serum IgG levels after a standard IVIG dose is associated with poor
prognosis. This parameter was not examined in our case.
In spite of those poor prognostic factors the patient survived the respiratory insufficiency. We consider the success to avoid respiratory
insufficiency in this patient was related to the proper management in the ICU when the calculated EGRIS score showed a high risk of developing
respiratory insufficiency, early decision to administer IVIg, and a relatively safer GBS type, AMAN.
Some studies suggest a second IVIg dose in not well-recovered patients. So, a second IVIg dose might be useful for this patient.
In GBS patients with poor prognostic factors, immediate treatment with IVIg as well as proper
Referene Conclusion

supportive management are beneficial for the patients. We think that low dose IVIg could
avoid a respiratory insufficiency in this patient but failed to show a dramatic improvement of
limb muscles strength. A repeated dose might be beneficial for this patient.
Access to PE or IVIg for GBS patients need to be improved in our country.
1. Tandel H, Vanza J, Pandya N, Jani P. Guillain Barre Syndrome. European journal of pharmaceutical and medical research. 2016. h.
366-371 Keywords: Guillain-Barre Syndrome, Acute
2. Winer J. An update in guillain barre syndrome Syndrome. Hindawi publishing corporation Autoimmune Disease. 2014 Motor Axonal Neuropathy, Low Dose IVIg
3. Doorn P, Kuitwaard K, Walgraard C, Koningsveld R, Ruts L, Jacobs B. IVIG Treatment and prognosis in Guillain barre syndrome. J clin
Immunol. 2010
4. Sinan A, Dogan M, Akgun C, Peker E, Sayn R, Aktar F. Clinical Features and Prognosis with Guillain Barre Syndrome. Ann Indian
Acad Neurology. 2011; 14(2): 98-102.