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Hypoglossal Nerve Palsy due to Compression by a Persistent Primitive Hypoglossal Artery: Case Report
ARTICLE IN PRESS
Case Report
Hypoglossal Nerve Palsy due to Compression by a Persistent Primitive Hypoglossal Artery: Case Report Hiroki Hikichi, MD,* Tatsuya Ueno, MD, PhD,* Masatoshi Iwamura, MD,† Haruo Nishijima, MD, PhD,‡ Akira Arai, MD, PhD,* Chieko Suzuki, MD, PhD,‡ Hiroshi Midorikawa, MD, PhD,† Jin-ichi Nunomura, MD, PhD,* and Masahiko Tomiyama, MD, PhD‡
A 51-year-old Japanese woman was admitted to our hospital because of speech difficulty following severe headache. Neurological examination showed dysarthria and tongue weakness on the right side, indicating right hypoglossal nerve palsy. Needle electromyography of the right side of the tongue showed fibrillation potentials. Magnetic resonance angiography and computed tomography angiography revealed a right, persistent, primitive hypoglossal artery (PPHA) that met Lie’s diagnostic criteria. Digital subtraction angiography showed an extended PPHA with irregular caliber in the portion running through the right hypoglossal canal. We diagnosed compression neuropathy of the hypoglossal nerve due to PPHA enlargement based on the findings of ipsilateral hypoglossal nerve palsy, fibrillation that indicated peripheral nerve palsy, and the enlarged diameter of the portion of the PPHA running through the right hypoglossal canal. We prescribed antihypertensive therapy. At 1 year after onset, her tongue weakness was alleviated. Clinicians should consider compression neuropathy due to a PPHA as one of the possibilities in the differential diagnosis of hypoglossal nerve palsy. Key Words: Hypoglossal nerve palsy—persistent primitive hypoglossal artery— compression neuropathy—neurovascular compression—vascular malformation © 2019 Elsevier Inc. All rights reserved.
Case Report A 51-year-old Japanese woman experienced acute onset of speech difficulty following severe headache. She presented From the *Department of Neurology, Aomori Prefectural Central Hospital, Aomori, Japan; †Department of Neuroendovascular Therapy, Aomori Prefectural Central Hospital, Aomori, Japan; and ‡Department of Neurology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan. Received July 17, 2019; revision received September 20, 2019; accepted October 2, 2019. Grant support: This research did not receive any specific grants from funding agencies in the public, commercial, or not-for-profit sectors. Address correspondence to Hiroki Hikichi, MD, Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-Tsukurimichi, Aomori 030-8551, Japan. E-mail: [email protected]. 1052-3057/$ - see front matter © 2019 Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.jstrokecerebrovasdis.2019.104459
to our hospital 2 months after onset. Her past medical history included hypertension with unremarkable family history. Neurological examination showed dysarthria and weakness on the tongue’s right side, indicating right hypoglossal nerve palsy (HNP; Fig 1A). She had no obvious sensory deficit, Horner syndrome signs, cerebellar ataxia, or pyramidal tract disorder. Two months after onset, needle electromyography showed right-tongue fibrillation potentials. Brain magnetic resonance imaging was normal (Fig 1B). MR angiography and computed tomography angiography revealed an atypical artery originating from the right internal carotid artery (ICA), entering the skull through the right hypoglossal canal, and extending as the basilar artery (Fig 1C,D). Digital subtraction angiography showed that the artery originated from the ICA at the first-to-second cervical vertebrae. Posterior communicating artery was absent. We identified a persistent primitive hypoglossal artery (PPHA).1 Digital subtraction
Journal of Stroke and Cerebrovascular Diseases, Vol. &&, No. && (&&), 2019: 104459
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ARTICLE IN PRESS H. HIKICHI ET AL.
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Figure 1. (A) On admission, neurological examination shows the patient’s tongue deviated to the right. (B) Fluid-attenuated inversion recovery image shows no abnormality in the medulla oblongata. (C) Magnetic resonance angiography reveals an atypical artery originating from the right internal carotid artery (arrow). (D) Computed tomography angiography shows the artery entering the skull through the right hypoglossal canal (arrow). (E) Digital subtraction angiography shows the extended persistent primitive hypoglossal artery with irregular caliber in the portion running through the right hypoglossal canal (arrow). (F) Right hypoglossal nerve palsy was alleviated 1 year after onset.
angiography showed 5-mm PPHA extension, with irregular caliber in the portion in the right hypoglossal canal (Fig 1E). We diagnosed compression neuropathy of the hypoglossal nerve caused by the enlarged PPHA. Antihypertensive therapy was prescribed. Her symptoms resolved 6 months after onset, and the tongue weakness was almost completely resolved by 1 year (Fig 1F).
Discussion The patient’s angiographic findings met Lie’s diagnostic criteria for PPHA.1 Although generally an incidental angiographic finding, PPHA has been reported to increase the risk of intracranial aneurysm.2 Also, an aneurysm of the PPHA stump and calcified PPHA have caused HNP.3,4 The patient’s headache at the onset suggested the possibility of dissection, but after recovery there were no obvious vascular changes on MR angiography (Supplementary Figure). Absence of neurological deficits (except HNP) and negative magnetic resonance imaging findings made brain stem infarction highly unlikely. A cross section of the patient’s PPHA in the hypoglossal canal
measured 5 mm, indicating enlargement,4 which might be due to its anomalous structures associated with hemodynamic stress caused by connection between the anterior and posterior circulations.5,6 Therefore, we believe that the enlarged PPHA might have caused compression neuropathy of the hypoglossal nerve because she exhibited ipsilateral HNP, fibrillation potentials of the tongue (indicating peripheral nerve palsy), and enlarged PPHA diameter in the right hypoglossal canal. In conclusion, PPHA may cause HNP. Thus, clinicians should consider compression neuropathy due to a PPHA in the differential diagnosis of HNP.
Informed Consent The patient provided written informed consent for publication of this report.
Conflicts of interest The authors have no conflicts of interest directly relevant to the content of this article.
ARTICLE IN PRESS HYPOGLOSSAL NERVE PALSY FROM NEUROVASCULAR COMPRESSION Acknowledgment: We thank Nancy Schatken, BS, MT (ASCP), from Edanz Group (www.edanzediting.com/ac), for editing a draft of this manuscript.
3.
Supplementary materials
4.
Supplementary material associated with this article can be found in the online version at doi:10.1016/j.jstrokecere brovasdis.2019.104459.
5.
References 1. Lie T, Hage J. Congenital anomalies of the carotid arteries. Plastic Reconstruct Surg 1968;42:283. 2. Wang M, Gu J, Lan P, et al. A persistent primitive hypoglossal artery as the sole supply to the brain associated
6.
3
with a basilar bifurcation aneurysm. Front Neurol 2017;8:168. Al-Memar A, Thrush D. Unilateral hypoglossal nerve palsy due to aneurysm of the stump of persistent hypoglossal artery. J Neurol Neurosurg Psychiatry 1998;64:405. Meila D, Wetter A, Brassel F, et al. Intermittent hypoglossal nerve palsy caused by a calcified persistent hypoglossal artery: an uncommon neurovascular compression syndrome. J Neurol Sci 2012;323:248-249. De Caro R, Parenti A, Munari PF. The persistent primitive hypoglossal artery: a rare anatomic variation with frequent clinical implications. Ann Anat = Anatomischer Anzeiger 1995;177:193-198. Varvari I, Bos EM, Dinkelaar W, et al. Fatal subarachnoid hemorrhage from an aneurysm of a persistent primitive hypoglossal artery: case series and literature overview. World Neurosurg 2018;117:285-291.
Case Report
Hypoglossal Nerve Palsy due to Compression by a Persistent Primitive Hypoglossal Artery: Case Report Hiroki Hikichi, MD,* Tatsuya Ueno, MD, PhD,* Masatoshi Iwamura, MD,† Haruo Nishijima, MD, PhD,‡ Akira Arai, MD, PhD,* Chieko Suzuki, MD, PhD,‡ Hiroshi Midorikawa, MD, PhD,† Jin-ichi Nunomura, MD, PhD,* and Masahiko Tomiyama, MD, PhD‡
A 51-year-old Japanese woman was admitted to our hospital because of speech difficulty following severe headache. Neurological examination showed dysarthria and tongue weakness on the right side, indicating right hypoglossal nerve palsy. Needle electromyography of the right side of the tongue showed fibrillation potentials. Magnetic resonance angiography and computed tomography angiography revealed a right, persistent, primitive hypoglossal artery (PPHA) that met Lie’s diagnostic criteria. Digital subtraction angiography showed an extended PPHA with irregular caliber in the portion running through the right hypoglossal canal. We diagnosed compression neuropathy of the hypoglossal nerve due to PPHA enlargement based on the findings of ipsilateral hypoglossal nerve palsy, fibrillation that indicated peripheral nerve palsy, and the enlarged diameter of the portion of the PPHA running through the right hypoglossal canal. We prescribed antihypertensive therapy. At 1 year after onset, her tongue weakness was alleviated. Clinicians should consider compression neuropathy due to a PPHA as one of the possibilities in the differential diagnosis of hypoglossal nerve palsy. Key Words: Hypoglossal nerve palsy—persistent primitive hypoglossal artery— compression neuropathy—neurovascular compression—vascular malformation © 2019 Elsevier Inc. All rights reserved.
Case Report A 51-year-old Japanese woman experienced acute onset of speech difficulty following severe headache. She presented From the *Department of Neurology, Aomori Prefectural Central Hospital, Aomori, Japan; †Department of Neuroendovascular Therapy, Aomori Prefectural Central Hospital, Aomori, Japan; and ‡Department of Neurology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan. Received July 17, 2019; revision received September 20, 2019; accepted October 2, 2019. Grant support: This research did not receive any specific grants from funding agencies in the public, commercial, or not-for-profit sectors. Address correspondence to Hiroki Hikichi, MD, Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-Tsukurimichi, Aomori 030-8551, Japan. E-mail: [email protected]. 1052-3057/$ - see front matter © 2019 Elsevier Inc. All rights reserved. https://doi.org/10.1016/j.jstrokecerebrovasdis.2019.104459
to our hospital 2 months after onset. Her past medical history included hypertension with unremarkable family history. Neurological examination showed dysarthria and weakness on the tongue’s right side, indicating right hypoglossal nerve palsy (HNP; Fig 1A). She had no obvious sensory deficit, Horner syndrome signs, cerebellar ataxia, or pyramidal tract disorder. Two months after onset, needle electromyography showed right-tongue fibrillation potentials. Brain magnetic resonance imaging was normal (Fig 1B). MR angiography and computed tomography angiography revealed an atypical artery originating from the right internal carotid artery (ICA), entering the skull through the right hypoglossal canal, and extending as the basilar artery (Fig 1C,D). Digital subtraction angiography showed that the artery originated from the ICA at the first-to-second cervical vertebrae. Posterior communicating artery was absent. We identified a persistent primitive hypoglossal artery (PPHA).1 Digital subtraction
Journal of Stroke and Cerebrovascular Diseases, Vol. &&, No. && (&&), 2019: 104459
1
ARTICLE IN PRESS H. HIKICHI ET AL.
2
Figure 1. (A) On admission, neurological examination shows the patient’s tongue deviated to the right. (B) Fluid-attenuated inversion recovery image shows no abnormality in the medulla oblongata. (C) Magnetic resonance angiography reveals an atypical artery originating from the right internal carotid artery (arrow). (D) Computed tomography angiography shows the artery entering the skull through the right hypoglossal canal (arrow). (E) Digital subtraction angiography shows the extended persistent primitive hypoglossal artery with irregular caliber in the portion running through the right hypoglossal canal (arrow). (F) Right hypoglossal nerve palsy was alleviated 1 year after onset.
angiography showed 5-mm PPHA extension, with irregular caliber in the portion in the right hypoglossal canal (Fig 1E). We diagnosed compression neuropathy of the hypoglossal nerve caused by the enlarged PPHA. Antihypertensive therapy was prescribed. Her symptoms resolved 6 months after onset, and the tongue weakness was almost completely resolved by 1 year (Fig 1F).
Discussion The patient’s angiographic findings met Lie’s diagnostic criteria for PPHA.1 Although generally an incidental angiographic finding, PPHA has been reported to increase the risk of intracranial aneurysm.2 Also, an aneurysm of the PPHA stump and calcified PPHA have caused HNP.3,4 The patient’s headache at the onset suggested the possibility of dissection, but after recovery there were no obvious vascular changes on MR angiography (Supplementary Figure). Absence of neurological deficits (except HNP) and negative magnetic resonance imaging findings made brain stem infarction highly unlikely. A cross section of the patient’s PPHA in the hypoglossal canal
measured 5 mm, indicating enlargement,4 which might be due to its anomalous structures associated with hemodynamic stress caused by connection between the anterior and posterior circulations.5,6 Therefore, we believe that the enlarged PPHA might have caused compression neuropathy of the hypoglossal nerve because she exhibited ipsilateral HNP, fibrillation potentials of the tongue (indicating peripheral nerve palsy), and enlarged PPHA diameter in the right hypoglossal canal. In conclusion, PPHA may cause HNP. Thus, clinicians should consider compression neuropathy due to a PPHA in the differential diagnosis of HNP.
Informed Consent The patient provided written informed consent for publication of this report.
Conflicts of interest The authors have no conflicts of interest directly relevant to the content of this article.
ARTICLE IN PRESS HYPOGLOSSAL NERVE PALSY FROM NEUROVASCULAR COMPRESSION Acknowledgment: We thank Nancy Schatken, BS, MT (ASCP), from Edanz Group (www.edanzediting.com/ac), for editing a draft of this manuscript.
3.
Supplementary materials
4.
Supplementary material associated with this article can be found in the online version at doi:10.1016/j.jstrokecere brovasdis.2019.104459.
5.
References 1. Lie T, Hage J. Congenital anomalies of the carotid arteries. Plastic Reconstruct Surg 1968;42:283. 2. Wang M, Gu J, Lan P, et al. A persistent primitive hypoglossal artery as the sole supply to the brain associated
6.
3
with a basilar bifurcation aneurysm. Front Neurol 2017;8:168. Al-Memar A, Thrush D. Unilateral hypoglossal nerve palsy due to aneurysm of the stump of persistent hypoglossal artery. J Neurol Neurosurg Psychiatry 1998;64:405. Meila D, Wetter A, Brassel F, et al. Intermittent hypoglossal nerve palsy caused by a calcified persistent hypoglossal artery: an uncommon neurovascular compression syndrome. J Neurol Sci 2012;323:248-249. De Caro R, Parenti A, Munari PF. The persistent primitive hypoglossal artery: a rare anatomic variation with frequent clinical implications. Ann Anat = Anatomischer Anzeiger 1995;177:193-198. Varvari I, Bos EM, Dinkelaar W, et al. Fatal subarachnoid hemorrhage from an aneurysm of a persistent primitive hypoglossal artery: case series and literature overview. World Neurosurg 2018;117:285-291.