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Neurology India, Vol. 58, No. 5, September-October, 2010, pp. 788-790 Letter to Editor An acute motor and sensory axonal neuropathy with cerebellar ataxia associated with anti-GD1b IgG and anti-GM1 IgG antibodies Zhirong Liu, Min Lou, Shanying Mao Department of Neurology, Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou - 310 009, China Correspondence Address: Date of Acceptance: 19-Jul-2010 Code Number: ni10218 PMID: 21045516 DOI: 10.4103/0028-3886.72173 Sir, The clinical features of Guillain-Barrι syndrome (GBS) tend to depend on the specificity of antiganglioside antibodies. [1] Serum anti-GD1b immunoglobulin G (IgG) antibodies are associated with the presence of ataxia in GBS, [2],[3] whereas serum anti-GM1 IgG antibodies are strongly associated with acute motor axonal neuropathies (AMAN). [4] In this report, we describe a patient with acute motor and sensory axonal neuropathy (AMSAN) and prominent cerebellar ataxia associated with the presence of anti-GD1b IgG and anti-GM1 IgG antibodies in the serum. A 59-year-old man was admitted with acute paresthesia, ataxia, and motor weakness of the limbs. Eight days prior to admission, he had 39ºC fever and a persistent cough for several days. Three days before admission, he developed paresthesia in the distal parts of both extremities. One day later, he experienced gait disturbance and developed slight weakness in both the lower extremities. On the day of admission, the unsteadiness was so severe that he could not walk independently. Neurological examination revealed insignificant motor weakness with absent deep tendon reflexes, both upper and lower limb ataxia, sensory loss in the glove and stocking distribution with no impairment of proprioceptive or vibratory sensation, and negative Romberg's sign. Electrophysiological examination showed normal distal motor latencies, motor conduction velocities, and sensory conduction velocities, but reduced sensory nerve action potential (SNAP) amplitudes (right median nerve: 4.6 μV; right ulnar nerve: 5.2 μV), and reduced compound muscle action potential (CMAP) amplitudes (right common peroneal nerve: 3.6 mV; right ulnar nerve: 4.0 mV). Serum was positive for anti-GD1b IgG and anti-GM1 IgG antibodies but negative for anti-GQ1b antibody. Cerebrospinal fluid (CSF) examination revealed no cells, protein of 104.6 mg/dl, and glucose of 55 mg/dl (blood sugar: 90 mg/dl). Cranial magnetic resonance imaging (MRI) scan was essentially normal. Serum vitamin B 12 and folic acid levels were normal The results of the human immunodeficiency virus and treponema pallidum particle agglutination assay (TPPA) tests were negative. He was treated with intravenous immunoglobulin (IVIG) for 5 days (0.4g/(kg· d). Over the following period, his ataxia improved. Ten days after treatment with IVIG, he could walk without assistance. Fifty days after the initial symptoms appeared, he had residual mild limb ataxia with normal muscle power and mild paresthesia in the fingers and toes. GBS is an acute immune-mediated inflammatory polyneuropathy that is characterized by a symmetrical flaccid paralysis with areflexia or hyporeflexia, distal paresthesias that are generally symmetrical, and disturbance of the autonomic nervous system. Subtypes of GBS include: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), AMAN, AMSAN, Miller-Fisher syndrome (FMS), and varieties that affect only the sensory or autonomic nervous systems. The types of GBS that produce ataxia include FMS, some types of sensory neuropathy, and cerebellar ataxic GBS; the latter, however, is very rare. In the present case, the presence of mild muscle weakness, distal paresthesia, areflexia, and cerebellar ataxia, which followed an upper respiratory infection, and the presence of protein-cytological dissociation in the CSF, led to the diagnosis of GBS. The electrophysiological examination was suggestive of axonal type of motor-sensory neuropathy. On the basis of these observations, we diagnosed this case as being a cerebellar ataxic variant of AMSAN. A large number of recent studies have shown that antiganglioside antibodies can play an important role in the pathophysiology of GBS and its variants. [1] Anti-GQ1b and anti-GT1a have been shown to be correlated with Fisher's syndrome, [5] and anti-GM1, anti-GD1a, anti-GM1b, and anti-GalNAc-GD1a IgG are associated with AMAN. [6] The anti-GD1b antibody binds to perinodal myelin, primary sensory neurons, and dorsal root ganglion cells. [7] It can exist in the cerebellar granular layer, the dentate nucleus, and the olivary nucleus. [8] An ataxic variant of GBS with positive anti-GD1b has been reported, and the ataxia was assumed to develop due to decreased deep sensation. [2] Our patient showed cerebellar ataxia rather than sensory ataxia. The coexistence of a prominent cerebellar involvement and AMSAN with positive anti-GD1b and anti-GM1 antibodies is extremely rare and our patient is probably the first such report. An ataxic variant of GBS can be misdiagnosed as FMS, acute cerebellitis, or acute cerebellar ataxia. [3] To differentiate between these clinical entities, it is useful to test for the presence of antibodies to GD1b and GQ1b IgG. A diagnosis of the ataxic variant of GBS is appropriate for cases with anti-GD1b and without anti-GQ1b, whereas the diagnosis of FMS can be made in cases where there is disturbance of extraocular movements with anti-GQ1b and without anti-GD1b. If a motor and sensory axonal neuropathy is present with cerebellar ataxia and positivity for serum anti-GM1 and anti-GD1b, a diagnosis of a cerebellar ataxic variant of AMSAN can be made. Acknowledgments This study was partly supported by grants from the Zhejiang Medical Science Research Fund of China (No. 2009A090). References
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