International Journal of Science and Research (IJSR) ISSN: 2319-7064 Index Copernicus Value (2016): 79.57 | Impact Factor (2018): 7.426 Volume 8 Issue 1, January 2019 www.ijsr.net Licensed Under Creative Commons Attribution CC BY An Overlapping Case of Miller Fisher Syndrome and Acute Motor Axonal Neuropathy in Children Kristopher May Pamudji 1 , Dewi Sutriani Mahalini 2 , IGN Made Suwarba 3 1, 2, 3 Department of Child Health Medical School, Udayana University/ Sanglah Hospital, Denpasar, Indonesia Abstract: Background : Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barre syndrome (GBS), classically characterized by a triad consist of ophtalmoplegia, ataxia, and areflexia. It is an immune-mediated polyneuropathy which may be preceded by mild respiratory or gastrointestinal infection. The diagnosis is based on clinical patterns, cerebrospinal fluid (CFS) analysis, and nerve conduction studies. Some severe variant forms like limb weakness (overlapping GBS), lower cranial nerves involvement (bulbar palsy), or central nervous system involvement (Bickerstaff encephalitis) have been reported, which might need proper diagnosis and treatment. Case Presentation : A 6 years old boy presented with drooping of the upper eyelid, imbalance and tendency to fall on either side while walking. The past medical history showed upper respiratory tract infection 1 week earlier. Physical examination showed complete ophthalmoplegia, ataxia, and lost of deep tendon reflex. The patient later developed mild lower limb weakness, difficulty in swallowing and bilateral facial nerve palsy. Cerebrospinal fluid analysis showed albuminocytologic dissociation and nerve conduction studies showed mix type lesion (axonal and demyelination), predominantly motor axonal lesion which support AMAN variant of GBS. Intravenous immunoglobulin (IVIG) was given and the symptoms gradually improved. The patient was discharged from hospital after 3 weeks with mild ophthalmoplegia and completely recovered after 6 weeks. Conclusion : We have reported an overlapping case of Miller Fisher syndrome and AMAN in 6 years old boy. Intravenous immunoglobulin therapy gave a good outcome in this case. Keywords: miller fisher syndrome, guillain barre syndrome, acute motor axonal neuropathy, ophtalmoplegia, ataxia. 1. Introduction Miller Fisher syndrome (MFS) is a rare variant of Guillain- Barre syndrome (GBS) which is characterized by a triad of ophthalmoplegia, ataxia and areflexia. It is observed in about 1%-5% of all GBS cases in Western countries and higher in Asia (19% in Taiwan and 25% in Japan) [1]-[4]. The annual incidence is about one patient per one million population [5]. This syndrome can be found in all ages, including infants, although it is less common in children than in adults. Male are more often affected than female in about 60%-68% [6]. The clinical triad of MFS mostly can be found together, but a purely ophthalmoplegic form and ophthalmoplegic with weakness or ataxia also exists. Ophthalmoplegia, whether occurring alone or with other parts, is almost associated with a specific antineural antibody, anti-GQ 1 b [6]-[8]. Glycans on lipooligosaccharides of preceding infectious organisms can induce anti-GQ 1 b antibodies that can bind to structurally identical glycan present on nerve gangliosides. This process can cause acute motor axonal neuropathy by antibody- mediated attack on the nerve axolemma driven by molecular mimicry between microbial and axolemmal surface molecules [9]. The titres of this anti-ganglioside antibody reach their peak at clinical presentation and decay rapidly in most cases concomitant with clinical recovery [10]. Diagnosis of MFS based on clinical features, but additional investigations can be helpful or even needed for confirmation [9], [11]. The presentation of clinical triad (ophthalmoplegia, ataxia and areflexia) in combination with absent or reduced sensory responses on clinical electrophysiology testing and elevated protein with a normal white blood cell in cerebrospinal fluid analysis could lead to the diagnosis of MFS [3]. We present an overlapping case of MFS and acute motor axonal neuropathy (AMAN) variant of GBS in children, which treated with intravenous immunoglobulin (IVIG). 2. Case A 6 years old boys whom previously healthy child, admitted to Sanglah Hospital Emergency Department with drooping of the upper eyelid since 2 days. The drooping did not deteriorate after being awake longer. The patient cannot move his eyes to every direction, but there is no blurred or double vision. The patient also had imbalance and tendency to fall on either side while walking. There is no headache nor decrease of consciousness. Bladder and bowel function were normal. The past medical history showed upper respiratory tract infection 1 week earlier. There was no recent history of trauma, drug abuse, alcohol addiction and vaccination. General physical examination was normal with stable vital signs. Cranial nerve examination revealed bilateral complete external ophthalmoplegia with ptosis that not improved with rest or ice pack test. The pupillary light reflex was sluggish in both eyes. Nystagmus was absent. Other cranial nerve examination showed normal result. Motor system examination showed normal muscle tone and power in both upper and lower limbs. There was no muscular wasting and involuntary movements. Deep tendon reflexes were absent in all four limbs with negative Babinski reflex. Sensations like thermal, pain and touch were normal. Rhomberg’s test was positive. He had ataxic gait with grossly impaired Tandem walking and tendency to fall on either side. Finger nose test and other cerebellar signs were normal. Laboratory examination revealed normal complete blood count. Initial cerebrospinal fluid (CSF) analysis at the fourth day of admission showed normal limit with white cell count Paper ID: ART20197391 10.21275/ART20197391 1778