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Capsular warning syndrome: report of a case. Acta Neurol Taiwan 2008;17:248–52. doi:http://dx.doi.org/10.1016/j.jocn.2011.12.030 Cranial nerve involvement as presenting sign of multifocal motor neuropathy Giuliana Galassi a,⇑ , Giovanna Albertini b , Franco Valzania a , Alberto Barbieri b a Department of Neurosciences, Via P. Giardini, 1350, University of Modena and Reggio Emilia, 41010 Modena, Italy b 2nd Intensive Care Unit, University of Modena & Reggio Emilia, Italy article info Article history: Received 12 October 2011 Accepted 28 December 2011 Keywords: Acute inflammatory demyelinating polyneuropathy Cranial nerve palsy Conduction block Multifocal motor neuropathy abstract Multifocal motor neuropathy (MMN) is characterized by slowly progressive, predominantly distal, asym- metric limb weakness and partial conduction blocks (CB) of motor axons. Cranial nerve involvement and respiratory failure are uncommon. We report two patients who exhibited unilateral hypoglossal and abducens palsy as presenting signs. Other remarkable features were autonomic instability and respira- tory failure due to bilateral phrenic nerve involvement. Treatment with intravenous (IV) immunoglobulin (Ig) resulted in an improvement. Patient 2, who showed IgM reactivity against ganglioside GM1, has been receiving maintenance therapy with IVIg for 7 years. We speculate that cranial weakness of our patients could be due to CB similar to those detected in the motor nerves of the extremities. Ó 2012 Elsevier Ltd. All rights reserved. 1. Introduction Multifocal motor neuropathy (MMN) is characterized by slowly progressive, asymmetric limb weakness, with minimal or no sensory symptoms. 1,2 Diagnostic criteria include a partial conduction block (CB) of motor axons at sites that are uncommon for compression or entrapment. 1,2 A CB is defined as equal or greater than 50% reduc- tion in the compound muscle action potential (CMAP) amplitude or at least 40% reduction in area between proximal and distal stimula- tion in nerves with a temporal dispersion of less than 30%. 1,2 Cranial nerve weakness is an uncommon presenting sign of MMN. 1–5 2. Case reports 2.1. Patient 1 A 64-year-old man was admitted after a 2-month history of hand cramping, shortness of breath, and abdominal distension. On admission, he presented left-sided tongue palsy with fascicula- tion, without pharyngeal weakness. He was able to walk; the upper and lower extremity strength was scored 4/5 proximally, and 3/5 distally on the Medical Research Council (MRC) scale. His deep re- flexes were weakened in the upper, and were absent in the lower limbs. Sensation was intact. Stool cultures excluded Campylobacter jejuni infection. Within 5 days, the weakness progressed to an asymmetric tetraparesis, with his distal strength graded between 1/5 and 2/5 in his upper and lower limbs. Anti-ganglioside GM1, 1,2 anti-acetylcholine receptor antibody assays (on days 9 and 15) were negative. Respiratory insufficiency required supple- mentary oxygen therapy and monitoring in the intensive care unit (ICU). Phrenic nerve involvement was confirmed by the bilateral absence of CMAP from diaphragm muscle. Cerebrospinal fluid (CSF) examination (days 5, 7, and 23) showed normal protein con- tent and no cells. Routine laboratory tests were unremarkable. Electrophysiology (days 5, 8, and 17) showed definite CB 1,2 at the axilla–elbow, elbow–wrist, and popliteal fossa–ankle segments of the median, ulnar, and tibial nerves. F-waves were not delayed, ex- cept in the tibial nerve. Distal latencies and velocity across seg- ments with CB were within normal limits. Sensory conduction studies of ulnar, median, and sural nerves were normal. The con- centric needle electromyography (EMG) demonstrated acute and chronic partial denervation in multiple muscles. The patient de- clined tongue and paraspinal EMG. Intravenous immunoglobulin (IVIg, 0.4 g/kg for 5 days) was given. He exhibited progressive recovery from the 3rd week and onward, becoming able to stand unaided. Eighteen weeks after initial onset, his tongue and extrem- ity weakness relapsed with the same intensity. An IVIg course was given monthly (0.4 g/kg for 5 days) for 6 months. Electrodiagnostic studies at 5 and 7 months confirmed CB in 6 of 8 tested motor nerves. Table 1 shows serial electrophysiological results for patient 1. Twenty eight weeks after onset, the patient was are- flexic with strength scored 3/5 proximally in the upper limbs, 1/5 in the wrist extensors, intrinsic hand, and feet muscles. His clinical condition worsened due to septic shock in the 8th month of his illness. 2.2. Patient 2 A 32-year-old woman awoke with horizontal diplopia on right lateral gaze and paresthesias in the dorsal aspects of her left lower limb and right hand. No preceding illnesses were reported. Neuro- logical evaluation revealed right-sided abducens and hypoglossal ⇑ Corresponding author. Tel.: +39 059 4225573; fax: 39 059 4223898. E-mail address: giulianagalassi@aliceposta.it (G. Galassi). Case Reports / Journal of Clinical Neuroscience 19 (2012) 1733–1735 1733