Acta Neurochir (2015) 157:1271–1273 DOI 10.1007/s00701-015-2442-4
LETTER TO THE EDITOR - BRAIN TUMORS
Unusual case of posterior fossa syndrome and bilateral hypertrophic olivary degeneration after surgical removal of a large fourth ventricle ependymoma in an adult Demian Manzano-Lopez Gonzalez 1 & Gerardo Conesa Bertran 1 & Jesus Lafuente Baraza 1
Received: 25 January 2015 / Accepted: 28 April 2015 / Published online: 9 May 2015 # Springer-Verlag Wien 2015
Dear Editor, Posterior fossa syndrome (PFS) is a complication of posterior fossa tumor surgery in children and occurs in 11 %–29 % of patients. PFS includes a wide range of complex neurobehavioral and motor symptoms. Cerebellar mutism is the most characteristic and commonly found. Recovery is spontaneous, but most patients will have language dysfunction and longterm sequelae [3, 5]. This syndrome has only occasionally been documented in adults [7]. Hypertrophic olivary degeneration (HOD) is a rare neurological disease caused by lesions in the Guillain-Mollaret triangle. This triangle is formed by the red nucleus, the ipsilateral inferior olivary nucleus, and the contralateral dentate nucleus [1, 6, 9, 10, 12]. HOD affects both, children and adults. It occurs relatively frequently after posterior fossa surgery in children affected by midline primary malignant tumors [11]. HOD may be unilateral or bilateral and is not always associated with an identificable lesional cause [2]. Palatal myoclonus is the most common symptom of HOD, although it is not present in all cases [2, 4, 12]. These complications of posterior fossa surgery are well known separately, but seldom mentioned coexisting together and, to the best of our knowledge, never in an adult patient. We present an exceptional case of an adult patient who underwent surgery on a large fourth ventricle ependymoma and developed symptoms consistent with PFS, also presenting with bilateral HOD. We also demonstrate the common cause for these neurological disorders.
* Demian Manzano-Lopez Gonzalez [email protected]; [email protected] 1
Department of Neurosurgery, Hospital del Mar. Parc de Salut Mar, Passeig Maritim 23-25, 08003 Barcelona, Spain
A 48-year-old female with no previous medical history presented with a 3-month history of headache, vomiting, and gait imbalance. On neurological examination the patient only showed upward gaze palsy. Cranial MRI showed a large fourth ventricle tumor with gadolinium enhancement that enlarged the ventricle and produced severe obstructive hydrocephalus, brain stem compression, and tonsillar herniation (Fig. 1a). The patient underwent a ventriculoperitoneal shunt insertion followed by a tumor resection as a second stage procedure. This was performed through a midline transvermian approach with intraoperative neurophysiological monitoring for the cranial nerves and long tracts. Gross total removal of the tumor was achieved. No affectation of the neurophysiological potentials was noted during surgery. The patient recovered well, and she only complained about diplopia caused by a bilateral 6th cranial nerve paresis. The lower cranial nerves were preserved. On the 3rd postoperative day the patient presented with mutism, apathy, unconcern, slow voluntary movements, and hypotonia. An MRI showed total removal of the tumor and brain stem decompression, and ruled out any complications (Fig. 1b and c). Two weeks later, the patient began to speak—first only words, then progressively she was able to construct simple sentences. However, the speech flow was severely affected, and neurobehavioral problems such as apathy, unconcern, and lack of initiative remained, as well as slow voluntary movements, hypotonia, and dysmetria. The patient was sent to a rehabilitation center and showed motor function improvement. The dysmetria diminished, and she was able to walk with almost no support. The frontal-like symptoms and speech problems persisted. Histopathological analysis confirmed grade 2 ependymoma. Six months after surgery, the patient presented with dysphagia, dysarthria, intention tremor, and gait worsening. The neurobehavioral problems remained
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Acta Neurochir (2015) 157:1271–1273
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Fig. 1 a Preoperative MRI showing a large fourth ventricle tumor producing severe obstructive hydrocephalus, brain stem compression, and tonsillar herniation. b Postoperative MRI at 3 days showing total removal of the tumor, brain stem decompression, and no complications. c Postoperative MRI at 3 days. Red arrow shows non injured olivary nuclei after decompression. d Postoperative MRI at 6 months. Red arrow shows affectation of both dentate nuclei areas bilaterally with hypersignal on T2-weighted images. e Postoperative MRI 6 months after
surgery. Red arrow shows bilateral HOD with hypersignal on T2weighted images on both olivary nuclei, predominantly on the right olivary nucleus. f Postoperative MRI 3 years after surgery still shows signs of bilateral HOD (red arrow) with high signal on T2-weighted images on both olivary nuclei. As in the previous MR studies, the images are bilateral with predominance on the right olivary nucleus. The hypersignal intensity is less than that observed on the previous MRI, but still clearly visible
the same. An MRI showed signs of bilateral HOD with hypersignal on T2-weighted images on both olivary and dentate nuclei bilaterally without gadolinium enhancement (Fig. 1d and e). The patient showed no palatal myoclonus, but the dysphagia and dysarthria worsened, as well as other motor symptoms, and the patient progressively lost autonomy. Two years after surgery, the patient needed a gastrostomy because of severe dysphagia, and she was no longer able to walk or manipulate objects because of severe gait disturbance, intention tremor, and dysmetria. However, she presented no palatal myoclonus. Three years after surgery, the patient still had disabling neurological sequelae. The MRI still showed signs of bilateral HOD (Fig. 1f). No tumor recurrence was detected. The most accepted explanation for PFS emphasizes the role of bilateral injury of the dentate nuclei. There is a bilateral interruption of the dentato-thalamo-cortical pathway; this produces a reversed cerebello-cerebral diaschisis phenomenon that leads to frontal lobe dysfunction and precipitates PFS. The dentate nucleus also forms part of the dentato-rubroolivary system (the Guillain-Mollaret triangle). Bilateral damage to the dentate nuclei may lead to bilateral HOD by a transsynaptic degeneration phenomenon [8]. Because of this anatomical overlap concerning the dentate nuclei (but by
different pathogenic mechanisms), PFS and bilateral HOD may develop, as demonstrated in the presented case. The presented case is consistent with both neurological entities, PFS and bilateral HOD, and to the best of our knowledge, this is the first reported case in an adult in which both pathologies, their causes, and their consequences are demonstrated. Conflicts of interest None Patient consent The patient and her next of kin consented to the submission of the case report for submission to the journal.
References 1.
Blanco Ulla M, Lopez Carballeira A, Pumar Cebreiro JM (2014) Magnetic resonance imaging of hypertrophic olivary degeneration. Radiología. doi:10.1016/j.rx.2014.12.008 2. Carr C, Hunt C, Kaufmann T, Kotsenas A (2015) Frequency of bilateral hypertrophic olivary degeneration in a large retrospective cohort. J Neuroimaging 25(2):289–295 3. Charalambides C, Dinopoulos A, Sgouros S (2 009) Neuropsychological sequelae and quality of life following treatment of posterior fossa ependumomas in children. Childs Nerv Syst 25:1313–1320
Acta Neurochir (2015) 157:1271–1273 4.
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8.
Goyal M, Versnick E, Tuite P, Saint Cyr J, Kucharczyk W, Montanera W, Willinsky R, Mikulis D (2000) Hypertrophic olivary degeneration: metaanalysis of the temporal evolution of MR findings. AJNR Am J Neuroradiol 21:1073–1077 Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K (2011) Cerebellar mutism: review of the literature. Childs Nerv Syst 27(3): 355–363 Hornyak M, Osborn AG, Coudwell WT (2008) Hypertrophic olivary degeneration after surgical removal of cavernous malformations of the brain stem: report of four cases and review of the literature. Acta Neurochir (Wien) 150:149–156 Marien P, De Smet HJ, Wijgerde E, Verhoeven J, Crols R, De Dyen P (2013) Posterior fossa syndrome in adults: a new case and comprehensive survey of the literature. Cortex 49:284–300 Patay Z, Enterkin J, Harreld JH, Yuan Y, Löbel U, Rumboldt Z, Khan R, Boop F (2014) MR imaging evaluation of inferior olivary
1273 nuclei: comparison of postoperative subjects with and without posterior fossa syndrome. AJNR Am J Neuroradiol 35(4):797–802 9. Sanverdi S, Oguz K, Haliloglu G (2012) Hypertrophic olivary degeneration in children: four new cases and a review of the literature with an emphasis on the MRI findings. Br J Radiol 85:511–516 10. 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 11. Tartaglione T, Izzo G, Alexandre A, Botto A, Maria Di Lella G, Gaudino S, Caldarelli M, Colosimo (2015) MRI findings of olivary degeneration after surgery for posterior fossa tumours in children: incidence, time course and correlation with tumour grading. Radiol Med 12. Yun J-H, Ahn JS, Park JC, Kwon DH, Kim CJ (2013) Hypertrophic olivary degeneration following surgical resection or gamma knife radiosurgery of brain cavernous malformations: an 11-case series and a review of literature. Acta Neurochir 155:469–476
LETTER TO THE EDITOR - BRAIN TUMORS
Unusual case of posterior fossa syndrome and bilateral hypertrophic olivary degeneration after surgical removal of a large fourth ventricle ependymoma in an adult Demian Manzano-Lopez Gonzalez 1 & Gerardo Conesa Bertran 1 & Jesus Lafuente Baraza 1
Received: 25 January 2015 / Accepted: 28 April 2015 / Published online: 9 May 2015 # Springer-Verlag Wien 2015
Dear Editor, Posterior fossa syndrome (PFS) is a complication of posterior fossa tumor surgery in children and occurs in 11 %–29 % of patients. PFS includes a wide range of complex neurobehavioral and motor symptoms. Cerebellar mutism is the most characteristic and commonly found. Recovery is spontaneous, but most patients will have language dysfunction and longterm sequelae [3, 5]. This syndrome has only occasionally been documented in adults [7]. Hypertrophic olivary degeneration (HOD) is a rare neurological disease caused by lesions in the Guillain-Mollaret triangle. This triangle is formed by the red nucleus, the ipsilateral inferior olivary nucleus, and the contralateral dentate nucleus [1, 6, 9, 10, 12]. HOD affects both, children and adults. It occurs relatively frequently after posterior fossa surgery in children affected by midline primary malignant tumors [11]. HOD may be unilateral or bilateral and is not always associated with an identificable lesional cause [2]. Palatal myoclonus is the most common symptom of HOD, although it is not present in all cases [2, 4, 12]. These complications of posterior fossa surgery are well known separately, but seldom mentioned coexisting together and, to the best of our knowledge, never in an adult patient. We present an exceptional case of an adult patient who underwent surgery on a large fourth ventricle ependymoma and developed symptoms consistent with PFS, also presenting with bilateral HOD. We also demonstrate the common cause for these neurological disorders.
* Demian Manzano-Lopez Gonzalez [email protected]; [email protected] 1
Department of Neurosurgery, Hospital del Mar. Parc de Salut Mar, Passeig Maritim 23-25, 08003 Barcelona, Spain
A 48-year-old female with no previous medical history presented with a 3-month history of headache, vomiting, and gait imbalance. On neurological examination the patient only showed upward gaze palsy. Cranial MRI showed a large fourth ventricle tumor with gadolinium enhancement that enlarged the ventricle and produced severe obstructive hydrocephalus, brain stem compression, and tonsillar herniation (Fig. 1a). The patient underwent a ventriculoperitoneal shunt insertion followed by a tumor resection as a second stage procedure. This was performed through a midline transvermian approach with intraoperative neurophysiological monitoring for the cranial nerves and long tracts. Gross total removal of the tumor was achieved. No affectation of the neurophysiological potentials was noted during surgery. The patient recovered well, and she only complained about diplopia caused by a bilateral 6th cranial nerve paresis. The lower cranial nerves were preserved. On the 3rd postoperative day the patient presented with mutism, apathy, unconcern, slow voluntary movements, and hypotonia. An MRI showed total removal of the tumor and brain stem decompression, and ruled out any complications (Fig. 1b and c). Two weeks later, the patient began to speak—first only words, then progressively she was able to construct simple sentences. However, the speech flow was severely affected, and neurobehavioral problems such as apathy, unconcern, and lack of initiative remained, as well as slow voluntary movements, hypotonia, and dysmetria. The patient was sent to a rehabilitation center and showed motor function improvement. The dysmetria diminished, and she was able to walk with almost no support. The frontal-like symptoms and speech problems persisted. Histopathological analysis confirmed grade 2 ependymoma. Six months after surgery, the patient presented with dysphagia, dysarthria, intention tremor, and gait worsening. The neurobehavioral problems remained
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Acta Neurochir (2015) 157:1271–1273
a
b
c
d
e
f
Fig. 1 a Preoperative MRI showing a large fourth ventricle tumor producing severe obstructive hydrocephalus, brain stem compression, and tonsillar herniation. b Postoperative MRI at 3 days showing total removal of the tumor, brain stem decompression, and no complications. c Postoperative MRI at 3 days. Red arrow shows non injured olivary nuclei after decompression. d Postoperative MRI at 6 months. Red arrow shows affectation of both dentate nuclei areas bilaterally with hypersignal on T2-weighted images. e Postoperative MRI 6 months after
surgery. Red arrow shows bilateral HOD with hypersignal on T2weighted images on both olivary nuclei, predominantly on the right olivary nucleus. f Postoperative MRI 3 years after surgery still shows signs of bilateral HOD (red arrow) with high signal on T2-weighted images on both olivary nuclei. As in the previous MR studies, the images are bilateral with predominance on the right olivary nucleus. The hypersignal intensity is less than that observed on the previous MRI, but still clearly visible
the same. An MRI showed signs of bilateral HOD with hypersignal on T2-weighted images on both olivary and dentate nuclei bilaterally without gadolinium enhancement (Fig. 1d and e). The patient showed no palatal myoclonus, but the dysphagia and dysarthria worsened, as well as other motor symptoms, and the patient progressively lost autonomy. Two years after surgery, the patient needed a gastrostomy because of severe dysphagia, and she was no longer able to walk or manipulate objects because of severe gait disturbance, intention tremor, and dysmetria. However, she presented no palatal myoclonus. Three years after surgery, the patient still had disabling neurological sequelae. The MRI still showed signs of bilateral HOD (Fig. 1f). No tumor recurrence was detected. The most accepted explanation for PFS emphasizes the role of bilateral injury of the dentate nuclei. There is a bilateral interruption of the dentato-thalamo-cortical pathway; this produces a reversed cerebello-cerebral diaschisis phenomenon that leads to frontal lobe dysfunction and precipitates PFS. The dentate nucleus also forms part of the dentato-rubroolivary system (the Guillain-Mollaret triangle). Bilateral damage to the dentate nuclei may lead to bilateral HOD by a transsynaptic degeneration phenomenon [8]. Because of this anatomical overlap concerning the dentate nuclei (but by
different pathogenic mechanisms), PFS and bilateral HOD may develop, as demonstrated in the presented case. The presented case is consistent with both neurological entities, PFS and bilateral HOD, and to the best of our knowledge, this is the first reported case in an adult in which both pathologies, their causes, and their consequences are demonstrated. Conflicts of interest None Patient consent The patient and her next of kin consented to the submission of the case report for submission to the journal.
References 1.
Blanco Ulla M, Lopez Carballeira A, Pumar Cebreiro JM (2014) Magnetic resonance imaging of hypertrophic olivary degeneration. Radiología. doi:10.1016/j.rx.2014.12.008 2. Carr C, Hunt C, Kaufmann T, Kotsenas A (2015) Frequency of bilateral hypertrophic olivary degeneration in a large retrospective cohort. J Neuroimaging 25(2):289–295 3. Charalambides C, Dinopoulos A, Sgouros S (2 009) Neuropsychological sequelae and quality of life following treatment of posterior fossa ependumomas in children. Childs Nerv Syst 25:1313–1320
Acta Neurochir (2015) 157:1271–1273 4.
5.
6.
7.
8.
Goyal M, Versnick E, Tuite P, Saint Cyr J, Kucharczyk W, Montanera W, Willinsky R, Mikulis D (2000) Hypertrophic olivary degeneration: metaanalysis of the temporal evolution of MR findings. AJNR Am J Neuroradiol 21:1073–1077 Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K (2011) Cerebellar mutism: review of the literature. Childs Nerv Syst 27(3): 355–363 Hornyak M, Osborn AG, Coudwell WT (2008) Hypertrophic olivary degeneration after surgical removal of cavernous malformations of the brain stem: report of four cases and review of the literature. Acta Neurochir (Wien) 150:149–156 Marien P, De Smet HJ, Wijgerde E, Verhoeven J, Crols R, De Dyen P (2013) Posterior fossa syndrome in adults: a new case and comprehensive survey of the literature. Cortex 49:284–300 Patay Z, Enterkin J, Harreld JH, Yuan Y, Löbel U, Rumboldt Z, Khan R, Boop F (2014) MR imaging evaluation of inferior olivary
1273 nuclei: comparison of postoperative subjects with and without posterior fossa syndrome. AJNR Am J Neuroradiol 35(4):797–802 9. Sanverdi S, Oguz K, Haliloglu G (2012) Hypertrophic olivary degeneration in children: four new cases and a review of the literature with an emphasis on the MRI findings. Br J Radiol 85:511–516 10. 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 11. Tartaglione T, Izzo G, Alexandre A, Botto A, Maria Di Lella G, Gaudino S, Caldarelli M, Colosimo (2015) MRI findings of olivary degeneration after surgery for posterior fossa tumours in children: incidence, time course and correlation with tumour grading. Radiol Med 12. Yun J-H, Ahn JS, Park JC, Kwon DH, Kim CJ (2013) Hypertrophic olivary degeneration following surgical resection or gamma knife radiosurgery of brain cavernous malformations: an 11-case series and a review of literature. Acta Neurochir 155:469–476
