Segmental Spinal Myoclonus and Metastatic Cervical Ganglioglioma: An Unusual Association

Abstract

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 suffering 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 pertinent literature to add some more information about the unusual association between spinal cord tumors and spinal myoclonus.

Myoclonus in the critically ill: Diagnosis, management, and clinical impact

“…Less frequent critical illnesses that can present with myoclonus are HIV encephalopathy (Canafoglia et al, 2003;Kanjanasut et al, 2010) and prion diseases, such as CreutzfeldtJakob disease (CJD) (Binelli et al, 2010;Chen et al, 2010). Myoclonus generated from spinal cord segments can be associated with traumatic spinal cord injury (Calancie, 2006), spinal cord tumors (Massimi et al, 2009), the Stiff-Person syndrome (Meinck et al, 1995), and with the progressive encephalomyelitis with rigidity and myoclonus (PERM) (Kraemer and Berlit, 2008;Kyskan et al, 2013;Shugaiv et al, 2013;Spitz et al, 2004).…”

“…By contrast, the less frequent, slowly repetitive spinal myoclonus is stimulus sensitive. In the ICU, spinal myoclonus can emerge in association with tumors (Massimi et al, 2009), trauma of spinal cord segments (Calancie, 2006), with spinal/intrathecal administration of anesthesia (Zamidei et al, 2010), or with autoimmune diseases including the Stiff-Person syndrome most commonly associated with GAD65 antibodies (Meinck et al, 1995) ( Table 2). Sometimes, the involved spinal segments spread in both directions rostrally and caudally, probably conducted through propriospinal tracts (Brown et al, 1994).…”

Spinal-generated movement disorders: a clinical review

“…One important clue is that in peripherally-generated myoclonus, the distribution of muscles involved corresponds to the nerve roots [ 7 , 8 ], plexus [ 9 ] or peripheral nerves [ 10 – 13 ], whereas SSM typically involves multiple muscles innervated by one to three adjacent spinal levels and can be bilateral. In SSM patients it is critical to exclude structural lesions of the spinal cord such as vascular, inflammatory [ 14 , 15 ], infectious [ 16 , 17 ], postinfectious [ 18 ], demyelinating [ 19 ], degenerative [ 20 , 21 ], paraneoplastic [ 22 ], and space-occupying lesions [ 23 , 24 ] with contrast MRI imaging. Specific treatment addresses the underlying etiology if one is found, while symptomatic treatments include clonazepam [ 21 , 25 , 26 ], valproic acid [ 26 ] and levetiracetam [ 27 ].…”

Can a Plegic Leg Move Itself?

“…No segundo dia de internamento foram identificados movimentos involuntários do membro inferior esquerdo, tendo sido solicitada observação urgente por Neurologia, na qual se apurou uma paraplegia espástica, hiperreflexia osteo-tendinosa nos membros inferiores, sinal de Babinski bilateral, nível sensitivo térmico-álgico * Contudo, há raros casos descritos na literatura de mioclonias segmentares estímulo-sensíveis. [3][4][5] e táctil por T5, compromisso propriocetivo nos membros inferiores e retenção urinária a requerer algaliação. Foram também objetivados movimentos involuntários do membro inferior esquerdo (Vídeo 1), que se iniciavam espontaneamente sem estímulo evidente, mas que eram facilmente despertáveis com pesquisa do reflexo cutâneo-plantar (Vídeo 2) e com avaliação do reflexo osteotendinoso rotuliano (Vídeo 3), amplos, rítmicos e estereotipados e que envolviam vários grupos musculares, similares a movimentos de pedalagem.…”

“…São vários os movimentos involuntários gerados ao nível medular, nomeadamente as mioclonias segmentares espinhais, as mioclonias proprio-espinhais, as discinésias paroxísticas secundárias e os movimentos de automatismo de pedalagem gerados por CPG, [1][2][3][4][5][6][7][8][9][10][11][12] cujas características fundamentais estão explicitadas na Tabela 1.…”