Acta Neurochir (2015) 157:885–887 DOI 10.1007/s00701-015-2385-9
LETTER TO THE EDITOR - PEDIATRICS
Bidirectional transynaptic degeneration after resection of brainstem pilocytic astrocytoma Ignacio Javier Gilete-Tejero & Mónica Rivero-Garvia & Manuel Royano-Sánchez & Francisco Javier Márquez-Rivas
Received: 6 January 2015 / Accepted: 23 February 2015 / Published online: 10 March 2015 # Springer-Verlag Wien 2015
Dear Editor, We would like to report the case of a 12-year-old boy who had undergone surgery for a brainstem pilocytic astrocytoma occupying the fourth ventricle (Fig. 1a). He underwent excision of the tumour by bilateral telovelar approach. Postoperative magnetic resonance imaging (MRI) showed dubious tumour remnants, with no other findings of interest (Fig. 1b) Although he had been discharged in good clinical condition, he was readmitted 4 months later due to a progressive ataxia, diplopia, dysarthria and left hemiparesis of 1 week’s evolution. After readmission, he experienced a deterioration of his neurological condition, with palatal and lingual paraesthesia, fasciculations in the lower limbs, myoclonus in the upper limbs, and a progressive deterioration of the level of consciousness. He underwent subsequent brain MRI, which showed hyperintense T2 lesions at the mesencephalic level in the red nucleus, presenting a clear progression in size (Fig. 1d, e), and a hyperintense T2 lesion with slight increase in volume and asymmetry in the right anterolateral region of the medulla (Fig. 1f), consistent with the theoretical position of the inferior olivary nucleus. In combination, these findings were highly suggestive of hypertrophic olivary degeneration (HOD). Even more strikingly, the serial MRI showed T1 hypointense images (Fig. 1g, h, i, j, k) and hyperintense on T2 images (not
I. J. Gilete-Tejero : M. Rivero-Garvia : F. J. Márquez-Rivas Paediatric Neurosurgery Unit, Neurosurgery Department, Virgen del Rocío and Virgen Macarena University Hospitals, Seville, Spain I. J. Gilete-Tejero (*) : M. Royano-Sánchez Department of Neurosurgery, Universitary Hospital Complex, Avenida de Elvas s/n., 06006 Badajoz, Spain e-mail: [email protected]
showed) at the bilateral dentatothalamocortical pathway level, consistent with ascending degeneration. The patient suffered a cardiorespiratory arrest and eventually died 3 weeks after being re-admitted. Neuronal degeneration secondary to the loss of afferent stimulation may be apparent in various areas of the nervous system, such as the optic pathways and the dorsal root [9, 10]. HOD is a form of transynaptic degeneration causing primarily hypertrophy rather than atrophy of the inferior olivary nucleus that occurs with a delayed fashion and secondarily to lesions with varied aetiologies occurring at the anatomical connections in the dentato-rubroolivary tract, also known as the Triangle of GuillainMollaret [2, 4–6, 8–10]. It contains a connection from the dentate nucleus to the ipsilateral red nucleus through the superior cerebellar peduncle. The fibres descend from the red nucleus through the central tegmental tract to connect to the inferior olivary nucleus. The inferior olivary nucleus sends its fibres through the inferior cerebellar peduncle towards the Purkinje fibres in the contralateral cerebellar cortex, which send their connections to the ipsilateral dentate nucleus, thereby closing the circuit (Fig. 1c). The most important clue for diagnosis is the detection of initial pathological changes in the dentato-rubro-olivary tract [9, 10] (the theoretical location of the red nucleus in our case). Symptoms commonly detailed are palatal tremor, Holmes tremor, ocular myoclonus, paraesthesia, ataxia, dysartria, diplopia, hemiparesis, low limb spasticity, hypoesthesia and dysmetria [9, 10]. In our case, the involvement of the dentatothalamocortical pathway is particularly important from the imaging perspective. Its anatomical relationships have been well defined due to its implications for cerebellar mutism [1, 3, 6, 7]. The efferent fibres originating in the dentate nucleus leave the
886
Acta Neurochir (2015) 157:885–887
Fig. 1 a Preoperative MRI revealed a tumour presenting an intense and heterogeneous uptake after administration of intravenous contrast and occupying the fourth ventricle. b Immediate postoperative MRI showed an image consistent with dubious tumour remnants (arrow) as the only finding of interest. c Illustration of the transneuronal association at the level of the Triangle of Guillain-Mollaret (original drawing by Manuel Royano Sánchez). The dentatothalamocortical pathway is also shown. In our case, a lesion at the mesencephalic tectum level in the theoretical anatomical location of the red nucleus appears to be the trigger for the
degeneration of both pathways. d, e Subsequent brain MRI performed during readmission showing hyperintense T2 lesions at the mesencephalic level, the theoretical location of the red nucleus, presenting a clear progression in size and f a hyperintense lesion (arrow) with a slight increase in volume and asymmetry in the right anterolateral bulbar region. The combination of these findings is highly suggestive of hypertrophic olivary degeneration. g, h, I, j, k Bilateral hypointense image in T1 at the level of the dentatothalamocortical pathway
cerebellum via the superior cerebellar peduncle, decussate at the mesencephalic tegmentum level, and establish synapses with the ventrolateral nucleus and ventral anterior nucleus of the thalamus. Meanwhile, post-synaptic neurons are projected and terminate at the level of extensive cortical regions, such as the primary motor cortex, premotor cortex and prefrontal cortex [3, 6] (Fig. 1c). The subsequent imaging tests enable us to speculate that a lesion to the mesencephalic tectum in the theoretical location of the red nucleus was the trigger for the degeneration of both pathways.
Current surgical strategies in the treatment of tumours on the posterior fossa aim to achieve the most extensive resections possible. Structures like the dentate nucleus, superior cerebellar peduncle and the mesencephalic tectum in particular are vulnerable to lesion due to surgical manipulations [3]. It is particularly important to recognise the consequences that damage to these structures and their physiopathological basis may entail, in order to minimise their potential impact. Acknowledgments The patient’s guardian has consented to the submission of the report of this case for publication.
Acta Neurochir (2015) 157:885–887 Conflicts of interest None.
Reference 1. Korah MP, Esiashvili N, Mazewski CM, Hudgins RJ, Tighiouart M, Janss AJ, Schwaibold FP, Crocker IR, Curan WJ Jr, Marcus RB Jr (2010) Incidence, risks an sequelae of posterior fossa syndrome in pediatric medulloblastoma. Int J Radiat Oncol, Biol, Phys 77:106–112 2. Marden FA (2013) Hypertrophic olivary degeneration due to pontine hemorrhage. JAMA Neurol 70:1330 3. Miller NG, Reddick WE, Kocak M, Glass JO, Löbel U, Morris B, Gajjar A, Patay Z (2010) Cerebellocerebral diaschisis is the likely mechanism of postsurgical posterior fossa syndrome in pediatric patients with midline cerebellar tumors. AJNR Am J Neuroradiol 31: 288–294 4. Nowak J, Alkonyi B, Rutkowski S, Homola GA, Warmuth-Metz M (2014) Hypertrophic olivary degeneration with gadolinium enhancement after posterior fossa surgery in a child with medulloblastoma. Child Nerv Syst 30:959–962
887 5. Orman G, Bosemani T, Jallo GI, Huisman TA, Poretti A (2014) Hypertrophic olivary degeneration in a child following midbrain tumor resection: longitudinal dffusion tensor imaging studies. J Neurosurg Pediatr 13:408–413 6. Otto J, Guenther P, Hoffmann KT (2013) Bileteral hypertrophic olivary degeneration in Wilson disease. Korean J Radiol 14:316–320 7. Pitsika M, Tsitouras V (2013) Cerebellar mutism. J Neurosurg Pediatr 12:604–614 8. Shinohara Y, Kinoshita T, Kinoshita F, Kaminou T, Watanabe T, Ogawa T (2013) Hypertrophic olivary degeneration after surgical resection of brain tumors. Acta Radiol 54:462–466 9. Tartaglione T, Izzo G, Alexandre A, Botto A, Di Lella GM, Gaudino S, Caldarelli M, Colosimo C (2015) MRI findings of olivary degeneration after surgery for posterior fossa tumours in children: incidence, time course and correlation with tumour grading. Radiol Med. doi:10.1007/S11547-014-047-x 10. Yun JH, Ahn JS, Park JC, Kwon DH, Kwun BD, Kim CJ (2013) Hypertrophic oivary degeneration following surgical resection or gamma knife radiosurgery of brainstem cavernous malformations: an 11-case series and a review of literature. Acta Neurochir (Wein) 155:469–476
LETTER TO THE EDITOR - PEDIATRICS
Bidirectional transynaptic degeneration after resection of brainstem pilocytic astrocytoma Ignacio Javier Gilete-Tejero & Mónica Rivero-Garvia & Manuel Royano-Sánchez & Francisco Javier Márquez-Rivas
Received: 6 January 2015 / Accepted: 23 February 2015 / Published online: 10 March 2015 # Springer-Verlag Wien 2015
Dear Editor, We would like to report the case of a 12-year-old boy who had undergone surgery for a brainstem pilocytic astrocytoma occupying the fourth ventricle (Fig. 1a). He underwent excision of the tumour by bilateral telovelar approach. Postoperative magnetic resonance imaging (MRI) showed dubious tumour remnants, with no other findings of interest (Fig. 1b) Although he had been discharged in good clinical condition, he was readmitted 4 months later due to a progressive ataxia, diplopia, dysarthria and left hemiparesis of 1 week’s evolution. After readmission, he experienced a deterioration of his neurological condition, with palatal and lingual paraesthesia, fasciculations in the lower limbs, myoclonus in the upper limbs, and a progressive deterioration of the level of consciousness. He underwent subsequent brain MRI, which showed hyperintense T2 lesions at the mesencephalic level in the red nucleus, presenting a clear progression in size (Fig. 1d, e), and a hyperintense T2 lesion with slight increase in volume and asymmetry in the right anterolateral region of the medulla (Fig. 1f), consistent with the theoretical position of the inferior olivary nucleus. In combination, these findings were highly suggestive of hypertrophic olivary degeneration (HOD). Even more strikingly, the serial MRI showed T1 hypointense images (Fig. 1g, h, i, j, k) and hyperintense on T2 images (not
I. J. Gilete-Tejero : M. Rivero-Garvia : F. J. Márquez-Rivas Paediatric Neurosurgery Unit, Neurosurgery Department, Virgen del Rocío and Virgen Macarena University Hospitals, Seville, Spain I. J. Gilete-Tejero (*) : M. Royano-Sánchez Department of Neurosurgery, Universitary Hospital Complex, Avenida de Elvas s/n., 06006 Badajoz, Spain e-mail: [email protected]
showed) at the bilateral dentatothalamocortical pathway level, consistent with ascending degeneration. The patient suffered a cardiorespiratory arrest and eventually died 3 weeks after being re-admitted. Neuronal degeneration secondary to the loss of afferent stimulation may be apparent in various areas of the nervous system, such as the optic pathways and the dorsal root [9, 10]. HOD is a form of transynaptic degeneration causing primarily hypertrophy rather than atrophy of the inferior olivary nucleus that occurs with a delayed fashion and secondarily to lesions with varied aetiologies occurring at the anatomical connections in the dentato-rubroolivary tract, also known as the Triangle of GuillainMollaret [2, 4–6, 8–10]. It contains a connection from the dentate nucleus to the ipsilateral red nucleus through the superior cerebellar peduncle. The fibres descend from the red nucleus through the central tegmental tract to connect to the inferior olivary nucleus. The inferior olivary nucleus sends its fibres through the inferior cerebellar peduncle towards the Purkinje fibres in the contralateral cerebellar cortex, which send their connections to the ipsilateral dentate nucleus, thereby closing the circuit (Fig. 1c). The most important clue for diagnosis is the detection of initial pathological changes in the dentato-rubro-olivary tract [9, 10] (the theoretical location of the red nucleus in our case). Symptoms commonly detailed are palatal tremor, Holmes tremor, ocular myoclonus, paraesthesia, ataxia, dysartria, diplopia, hemiparesis, low limb spasticity, hypoesthesia and dysmetria [9, 10]. In our case, the involvement of the dentatothalamocortical pathway is particularly important from the imaging perspective. Its anatomical relationships have been well defined due to its implications for cerebellar mutism [1, 3, 6, 7]. The efferent fibres originating in the dentate nucleus leave the
886
Acta Neurochir (2015) 157:885–887
Fig. 1 a Preoperative MRI revealed a tumour presenting an intense and heterogeneous uptake after administration of intravenous contrast and occupying the fourth ventricle. b Immediate postoperative MRI showed an image consistent with dubious tumour remnants (arrow) as the only finding of interest. c Illustration of the transneuronal association at the level of the Triangle of Guillain-Mollaret (original drawing by Manuel Royano Sánchez). The dentatothalamocortical pathway is also shown. In our case, a lesion at the mesencephalic tectum level in the theoretical anatomical location of the red nucleus appears to be the trigger for the
degeneration of both pathways. d, e Subsequent brain MRI performed during readmission showing hyperintense T2 lesions at the mesencephalic level, the theoretical location of the red nucleus, presenting a clear progression in size and f a hyperintense lesion (arrow) with a slight increase in volume and asymmetry in the right anterolateral bulbar region. The combination of these findings is highly suggestive of hypertrophic olivary degeneration. g, h, I, j, k Bilateral hypointense image in T1 at the level of the dentatothalamocortical pathway
cerebellum via the superior cerebellar peduncle, decussate at the mesencephalic tegmentum level, and establish synapses with the ventrolateral nucleus and ventral anterior nucleus of the thalamus. Meanwhile, post-synaptic neurons are projected and terminate at the level of extensive cortical regions, such as the primary motor cortex, premotor cortex and prefrontal cortex [3, 6] (Fig. 1c). The subsequent imaging tests enable us to speculate that a lesion to the mesencephalic tectum in the theoretical location of the red nucleus was the trigger for the degeneration of both pathways.
Current surgical strategies in the treatment of tumours on the posterior fossa aim to achieve the most extensive resections possible. Structures like the dentate nucleus, superior cerebellar peduncle and the mesencephalic tectum in particular are vulnerable to lesion due to surgical manipulations [3]. It is particularly important to recognise the consequences that damage to these structures and their physiopathological basis may entail, in order to minimise their potential impact. Acknowledgments The patient’s guardian has consented to the submission of the report of this case for publication.
Acta Neurochir (2015) 157:885–887 Conflicts of interest None.
Reference 1. Korah MP, Esiashvili N, Mazewski CM, Hudgins RJ, Tighiouart M, Janss AJ, Schwaibold FP, Crocker IR, Curan WJ Jr, Marcus RB Jr (2010) Incidence, risks an sequelae of posterior fossa syndrome in pediatric medulloblastoma. Int J Radiat Oncol, Biol, Phys 77:106–112 2. Marden FA (2013) Hypertrophic olivary degeneration due to pontine hemorrhage. JAMA Neurol 70:1330 3. Miller NG, Reddick WE, Kocak M, Glass JO, Löbel U, Morris B, Gajjar A, Patay Z (2010) Cerebellocerebral diaschisis is the likely mechanism of postsurgical posterior fossa syndrome in pediatric patients with midline cerebellar tumors. AJNR Am J Neuroradiol 31: 288–294 4. Nowak J, Alkonyi B, Rutkowski S, Homola GA, Warmuth-Metz M (2014) Hypertrophic olivary degeneration with gadolinium enhancement after posterior fossa surgery in a child with medulloblastoma. Child Nerv Syst 30:959–962
887 5. Orman G, Bosemani T, Jallo GI, Huisman TA, Poretti A (2014) Hypertrophic olivary degeneration in a child following midbrain tumor resection: longitudinal dffusion tensor imaging studies. J Neurosurg Pediatr 13:408–413 6. Otto J, Guenther P, Hoffmann KT (2013) Bileteral hypertrophic olivary degeneration in Wilson disease. Korean J Radiol 14:316–320 7. Pitsika M, Tsitouras V (2013) Cerebellar mutism. J Neurosurg Pediatr 12:604–614 8. Shinohara Y, Kinoshita T, Kinoshita F, Kaminou T, Watanabe T, Ogawa T (2013) Hypertrophic olivary degeneration after surgical resection of brain tumors. Acta Radiol 54:462–466 9. Tartaglione T, Izzo G, Alexandre A, Botto A, Di Lella GM, Gaudino S, Caldarelli M, Colosimo C (2015) MRI findings of olivary degeneration after surgery for posterior fossa tumours in children: incidence, time course and correlation with tumour grading. Radiol Med. doi:10.1007/S11547-014-047-x 10. Yun JH, Ahn JS, Park JC, Kwon DH, Kwun BD, Kim CJ (2013) Hypertrophic oivary degeneration following surgical resection or gamma knife radiosurgery of brainstem cavernous malformations: an 11-case series and a review of literature. Acta Neurochir (Wein) 155:469–476
