Neurol Sci (2015) 36:483–484 DOI 10.1007/s10072-014-1977-0
LETTER TO THE EDITOR
Embolization treatment of cerebral arteriovenous malformations and amyotrophic lateral sclerosis Ourania Katsavarou • Stefanos Ntampos • Ioannis Sarmas • Nikos Triantafyllou • Sotirios Giannopoulos • Athanassios P. Kyritsis
Received: 27 August 2014 / Accepted: 30 September 2014 / Published online: 7 October 2014 Ó Springer-Verlag Italia 2014
Keywords
AVM ALS Embolization
Dear Editor, Recently, cases of amyotrophic lateral sclerosis (ALS) have been reported after embolization of cerebral arteriovenous malformation (AVM). In a recent study that included 1,114 patients who were embolized for a cerebral AVM, seven cases that developed ALS, after a mean latency period of 14 years, were reported [1]. In all cases the initial limb of ALS symptom onset was ipsilateral to the AVM. All these patients had a rare AVM architecture with significant perinidal angiogenesis and received at least three embolization sessions. These AVM patients that developed ALS had lower vascular endothelial growth factor (VEGF) serum levels than controls [1]. Herein, we are reporting the possible association between ALS and AVM embolization, based on two cases that we encountered in our institute. There were two female patients (aged 45 and 36-year-old, respectively) that developed ALS after a latency period of 11 and 14 years from the first embolization session. These two patients received 3 and 4 embolization sessions, respectively
(Fig. 1). Contrary to Valavanis et al. in both patients that we encountered the initial limb of ALS symptom onset was contralateral to the AVM and there was gradual worsening of spasticity and paresis. Both patients had signs of upper and lower neuron degeneration and the neurophysiological features, such as signs of active and chronic denervation (fibrillation potentials, positive sharp waves and large motor unit potentials of increased duration) suggested ALS (Fig. 2). The first patient died 2 years after the onset of ALS symptoms, whereas the second patient is still alive, nearly 2 year after ALS diagnosis, but is dependent on a wheelchair with excessive spasticity and severe paraparesis. We believe that clinicians should be aware of this possible association and ALS occurrence should be further investigated in AVM patients that received embolization treatment. VEGF has been reported to be a modifier of ALS [2], unfortunately the method is not available in our Institution, and thus we have not tested our patients. VEGF has been shown to protect neurons from cell death, whereas VEGF gene or protein treatment showed to prolong the survival of ALS rodent models [2], activating a number of different intracellular signaling pathways that have been
O. Katsavarou (&) S. Ntampos I. Sarmas S. Giannopoulos A. P. Kyritsis Department of Neurology, University of Ioannina School of Medicine, 45110 Ioannina, Greece e-mail: [email protected] N. Triantafyllou First Department of Neurology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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Neurol Sci (2015) 36:483–484
Fig. 1 a Gadolinium-enhanced, axial T1-weighted image showing an arteriovenous malformation of the right cerebral hemisphere, in a 42-year-old woman that was treated with 3 embolization sessions. b Axial T2-weighted MRI demonstrating large draining veins (arrow)
the existing literature we support the hypothesis of a possible connection between embolized AVMs and ALS. If the reduction of VEGF after embolization unmasks ALS needs further research. Conflict of interest
None.
References
Fig. 2 Electromyography recordings of the 36-year-old female, demonstrating spontaneous activity of the right first dorsal interosseous muscle
implicated in promoting neuroprotection [3]. Apart from VEGF, other well-characterized neuron trophic factors can be investigated [3]. In conclusion adding these two cases to
123
1. Valavanis A, Schwarz U, Baumann CR, Weller M, Linnebank M (2014) Amyotrophic lateral sclerosis after embolization of cerebral arterioveneous malformations. J Neurol 261:732–737 2. Lambrechts D, Storkebaum E, Morimoto M et al (2003) VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death. Nat Genet 34:383–394 3. Tovar-Y-Romo LB, Ramı´rez-Jarquı´n UN, Lazo-Go´mez R, Tapia R (2014) Trophic factors as modulators of motor neuron physiology and survival: implications for ALS therapy. Front Cell Neurosci 28:61
LETTER TO THE EDITOR
Embolization treatment of cerebral arteriovenous malformations and amyotrophic lateral sclerosis Ourania Katsavarou • Stefanos Ntampos • Ioannis Sarmas • Nikos Triantafyllou • Sotirios Giannopoulos • Athanassios P. Kyritsis
Received: 27 August 2014 / Accepted: 30 September 2014 / Published online: 7 October 2014 Ó Springer-Verlag Italia 2014
Keywords
AVM ALS Embolization
Dear Editor, Recently, cases of amyotrophic lateral sclerosis (ALS) have been reported after embolization of cerebral arteriovenous malformation (AVM). In a recent study that included 1,114 patients who were embolized for a cerebral AVM, seven cases that developed ALS, after a mean latency period of 14 years, were reported [1]. In all cases the initial limb of ALS symptom onset was ipsilateral to the AVM. All these patients had a rare AVM architecture with significant perinidal angiogenesis and received at least three embolization sessions. These AVM patients that developed ALS had lower vascular endothelial growth factor (VEGF) serum levels than controls [1]. Herein, we are reporting the possible association between ALS and AVM embolization, based on two cases that we encountered in our institute. There were two female patients (aged 45 and 36-year-old, respectively) that developed ALS after a latency period of 11 and 14 years from the first embolization session. These two patients received 3 and 4 embolization sessions, respectively
(Fig. 1). Contrary to Valavanis et al. in both patients that we encountered the initial limb of ALS symptom onset was contralateral to the AVM and there was gradual worsening of spasticity and paresis. Both patients had signs of upper and lower neuron degeneration and the neurophysiological features, such as signs of active and chronic denervation (fibrillation potentials, positive sharp waves and large motor unit potentials of increased duration) suggested ALS (Fig. 2). The first patient died 2 years after the onset of ALS symptoms, whereas the second patient is still alive, nearly 2 year after ALS diagnosis, but is dependent on a wheelchair with excessive spasticity and severe paraparesis. We believe that clinicians should be aware of this possible association and ALS occurrence should be further investigated in AVM patients that received embolization treatment. VEGF has been reported to be a modifier of ALS [2], unfortunately the method is not available in our Institution, and thus we have not tested our patients. VEGF has been shown to protect neurons from cell death, whereas VEGF gene or protein treatment showed to prolong the survival of ALS rodent models [2], activating a number of different intracellular signaling pathways that have been
O. Katsavarou (&) S. Ntampos I. Sarmas S. Giannopoulos A. P. Kyritsis Department of Neurology, University of Ioannina School of Medicine, 45110 Ioannina, Greece e-mail: [email protected] N. Triantafyllou First Department of Neurology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
123
484
Neurol Sci (2015) 36:483–484
Fig. 1 a Gadolinium-enhanced, axial T1-weighted image showing an arteriovenous malformation of the right cerebral hemisphere, in a 42-year-old woman that was treated with 3 embolization sessions. b Axial T2-weighted MRI demonstrating large draining veins (arrow)
the existing literature we support the hypothesis of a possible connection between embolized AVMs and ALS. If the reduction of VEGF after embolization unmasks ALS needs further research. Conflict of interest
None.
References
Fig. 2 Electromyography recordings of the 36-year-old female, demonstrating spontaneous activity of the right first dorsal interosseous muscle
implicated in promoting neuroprotection [3]. Apart from VEGF, other well-characterized neuron trophic factors can be investigated [3]. In conclusion adding these two cases to
123
1. Valavanis A, Schwarz U, Baumann CR, Weller M, Linnebank M (2014) Amyotrophic lateral sclerosis after embolization of cerebral arterioveneous malformations. J Neurol 261:732–737 2. Lambrechts D, Storkebaum E, Morimoto M et al (2003) VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death. Nat Genet 34:383–394 3. Tovar-Y-Romo LB, Ramı´rez-Jarquı´n UN, Lazo-Go´mez R, Tapia R (2014) Trophic factors as modulators of motor neuron physiology and survival: implications for ALS therapy. Front Cell Neurosci 28:61
