Brief Communication Segmental Spinal Myoclonus and Metastatic Cervical Ganglioglioma: An Unusual Association Luca Massimi, MD, Domenica Battaglia, MD, Giovanna Paternoster, MD, Diego Martinelli, MD, Carmelo Sturiale, MD, and Concezio Di Rocco, MD Segmental spinal myoclonus rarely occurs in association with spinal cord tumor. Only 3 cases have been reported in children so far, mainly concerning astrocytomas of the thoracic spinal cord. We report on a 2-year-old boy suffer- ing from segmental spinal myoclonus involving the upper limbs and harboring a cervical tumor. The clinical and electrophysiological features ruled out a myoclonus of different origin (cortical, subcortical, propriospinal) other than other types of movement disorders. Neuroimaging and histological examinations showed the exceptional presence of a ganglioglioma as the cause of the segmental spinal myoclonus. The clinical and electrophysiological characteristics as well as the possible etiopathogenesis and differential diagnosis are discussed on the basis of the per- tinent literature to add some more information about the unusual association between spinal cord tumors and spinal myoclonus. Keywords: segmental spinal myoclonus; spinal cord tumor; ganglioglioma S ymptomatic spinal myoclonus usually results from degenerative, ischemic, metabolic, and infectious diseases or from toxic, traumatic, and surgical inju- ries. 1 Spinal cord tumors represent an unusual cause of spinal myoclonus, being sporadically reported in adults and/or mainly before the computed tomography (CT) or magnetic resonance imaging (MRI) era. 2,3 In children, such an association is very uncommon and limited to thor- acic astrocytomas. 4 We report on the ‘‘exceptional’’ case of a child harboring a cervical ganglioglioma presenting with segmental spinal myoclonus (SSM). The pertinent litera- ture is reviewed. Case Report This 2-year-old boy was admitted to our department after a 6-month history of upper limbs jerks. Transitory myoclonic jerks worsened by emotions were observed dur- ing the first year of life; no other neurological signs were present. Afterward, myoclonic jerks progressively extended up to include muscles of both upper limbs bilat- erally, and increased in frequency. The neurological exam- ination at the admission pointed out involuntary, spontaneous, synchronous myoclonic jerks of both arms and hands (left side > right), with a forced adduction and intrarotation of the arms. The myoclonus increased with emotional excitement and disappeared during sleep. Left arm weakness, reduction of pendular synkinesia, neck stiffness, and left Babinski sign were also evident. Video-electroencephalogram (EEG) recordings and simultaneous surface electromyogram (EMG) of the right and left deltoid, flexor radialis carpis, extensor radialis carpis, and quadriceps were performed. Electroencepha- lograms showed a normal awake activity and nonrapid eye movement sleep organization. Electromyogram demon- strated myoclonic jerks of the upper limbs (Figure 1A). The myoclonic activity was bilateral and rhythmic (steady state 60/min during wake, burst duration 150-300 ms). The myoclonus was synchronous in all the examined mus- cles. The jerks amplitude was 100 to 300 mV (left side pre- dominant). The myoclonus was continuous and was not modified by passive movements, voluntary activity, pos- tural changes, mental activity, sensitive or proprioceptive stimuli. The jerks frequency decreased with drowsiness up to 40/min and disappeared during the slow sleep (Figure 1B). Received May 6, 2008. Accepted for publication June 26, 2008. From the Pediatric Neurosurgery (LM, GP, CS, CDR) and Pediatric Neurology Department, (DB, DM), Catholic University Medical School, Rome, Italy. The authors have no conflicts of interest to disclose with regard to this article. Address correspondence to: Luca Massimi, Institute of Neurosurgery- Pediatric Neurosurgery, Largo A Gemelli, 8, 00168, Rome, Italy; e-mail: lmassimi@email.it. Massimi L, Battaglia D, Paternoster G, Martinelli D, Sturiale C, Di Rocco C. Segmental spinal myoclonus and metastatic cervical ganglioglioma: an unusual association. J Child Neurol. 2009;24:365-369. 365 Journal of Child Neurology Volume 24 Number 3 March 2009 365-369 # 2009 Sage Publications 10.1177/0883073808323027 http://jcn.sagepub.com hosted at http://online.sagepub.com at SWETS WISE ONLINE CONTENT on March 19, 2009 http://jcn.sagepub.com Downloaded from