Laing early onset distal myopathy 8 Bonnemann CG, Laing NG. Myopathies resulting from mutations in sarcomeric proteins. Curr Opin Neurol 2004;17:529–37. 9 Richard P, Charron P, Carrier L, for the EUROGENE Heart Failure Project, et al. Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy. Circulation 2003;107:2227–32. 10 Gowers WR. Myopathy and a distal form. BMJ, 1902;ii, 89–92. 11 Tajsharghi H, Thornell L-E, Lindberg C, et al. Myosin storage myopathy associated with a heterozygous missense mutations in MYH7. Ann Neurol 2003;54:494–500. 215 12 Fananapazir L, Dalakas MC, Cyran F, et al. Missense mutations in the bmyosin heavy-chain gene cause central core disease in hypertrophic cardiomyopathy. Proc Natl Acad Sci USA 1993;90:3993–7. 13 Caforio ALP, Rossi B, Risaliti R, et al. Type 1 fiber abnormalities in skeletal muscle of patients with hypertrophic and dilated cardiomyopathy: evidence of subclinical myogenic myopathy. J Am Coll Cardiol 1989;14:1464–73. 14 Smith ER, Heffernan LP, Sangalang VE, et al. Voluntary muscle involvement in hypertrophic cardiomyopathy. Ann Intern Med 1976;85:566–72. 15 Mastaglia FL, Phillips BA, Cala LA, et al. Early onset chromosome 14 linked distal myopathy (Laing). Neuromusc Disord 2001;12:350–7. NEUROLOGICAL PICTURE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . doi: 10.1136/jnnp.2005.071522 Medial medullary infarction A 60 year old man with hypertension and diabetes mellitus sought neurological consultation for sudden onset of numbness over the left side of body. On examination, he was conscious and had dysarthria. Right lingual paresis was observed on tongue protrusion (fig 1A). The other neurological findings included left lemniscal sensory impairment and mild left haemiparesis without facial involvement (fig 1B). The presence of crossed neurological signs—right (ipsilateral) hypoglossal palsy and left (contralateral) sensorimotor haemiparesis (with facial sparing)—localised the lesion to right medial medulla. Cranial magnetic resonance imaging (MRI) confirmed the clinical localisation (fig 2A, B). Intracranial portion of right vertebral artery was not visualised on the magnetic resonance angiogram (fig 2C). The topography of the lesion and the absence of flow in the right vertebral artery favoured infarction over demyelination as the likely aetiology. The neurological problem was ascribed to right medial medullary infarction due to occlusion of antero-medial medullary artery, originating from right vertebral artery. He eventually made good clinical recovery. Our patient presented with the classical clinical triad of Dejerine’s syndrome, that includes ipsilateral hypoglossal palsy, contralateral haemiparesis, and lemniscal sensory loss.1 Involvement of right hypoglossal nucleus and nerve fibres in the dorsomedial portion of upper medulla, right pyramid, Figure 1 (A) Right hypoglossal palsy elicited on tongue protrusion; (B) Left pronator drift evident on forward arm abduction. and medial lemniscus rostral to the motor and sensory decussations contributed to the observed neurological signs. Intracranial vertebral artery occlusion more often manifests with lateral medullary syndrome. Rarely does it produce medial medullary infarction as observed in our patient. Acknowledgements The authors thank Mr Rajesh for technical assistance. R Nandhagopal, S G Krishnamoorthy Department of Neurology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India D Srinivas Department of Radiology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India Correspondence to: Dr R Nandhagopal, Department of Neurology, Sri Venkateswara Institute of Medical Sciences, TIRUPATI-517 507, Andhra Pradesh, India; rnandagopal@yahoo.com Competing interests: none declared Consent has been obtained for figure 1. Reference 1 Bassetti C, Bogousslavsky J, Mattle H, et al. Medial medullary stroke: Report of seven patients and review of the literature. Neurology 1997;48:882–90. Figure 2 (A) Right medial medullary infarction seen on the T2 weighted coronal magnetic resonance imaging; (B) The same lesion in transaxial fluid attenuated inversion recovery sequence; (C) Magnetic resonance angiogram showing normal flow in the left vertebral artery (arrow). Note the absence of flow in the right vertebral artery (cross). www.jnnp.com