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Reversible pseudoathetosis and sensory ataxic gait caused by cervical spondylotic myelopathy
Journal of Clinical Neuroscience xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Reversible pseudoathetosis and sensory ataxic gait caused by cervical spondylotic myelopathy Wen-Juh Hwang ⇑ Department of Neurology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, 138 Sheng-Li Road, Tainan 704, Taiwan
a r t i c l e
i n f o
Article history: Received 5 June 2016 Accepted 1 August 2016 Available online xxxx Keywords: Cervical spondylotic myelopathy Pseudoathetosis Sensory ataxic gait
a b s t r a c t Proprioceptive deafferentation of spinal cord origin can cause pseudoathetosis, sensory ataxic gait, or both. The co-existence of pseudoathetosis and sensory ataxic gait caused by a surgically treatable condition of the spinal cord has been rarely reported. An 80-year-old man with cervical spondylotic myelopathy presented with severe sensory ataxic gait which confined him to a wheelchair. He also had poor control of his hands due to the pseudoathetoid movements of the fingers, which prevented him from sustaining constant muscle contraction. He underwent C3-4 and C4-5 anterior discectomies and anterior fusion. His neurological deficits gradually improved after the decompressive surgery. About 7 months postoperatively, he was totally independent in activities of daily living and needed no mobility aid. This case highlights the clinical importance of recognizing a surgically treatable and reversible condition of the spinal cord that causes pseudoathetosis and sensory ataxic gait. Ó 2016 Elsevier Ltd. All rights reserved.
1. Introduction Athetosis refers to an involuntary, slow, writhing movement primarily affecting the distal parts of the extremities. Pseudoathetosis is clinically indistinguishable from athetosis and is caused by impaired proprioception. Pseudoathetosis can occur with lesions located anywhere along the proprioceptive sensory pathways from the peripheral nerves to the parietal cortex [1–5]. While the exact mechanism of pseudoathetosis is uncertain, it is hypothesized that it occurs because of the failure of the striatum to properly integrate cortical motor and sensory inputs [2]. I report a patient with cervical spondylotic myelopathy with severe deafferentation of the four limbs that manifested as sensory ataxic gait and pseudoathetosis in the fingers. His neurological deficits markedly improved after surgical decompression. 2. Case report An 80-year-old man presented with poor control of his hands, paresthesia of the four limbs, and progressive unsteady gait for the previous 6 months. He had a medical history of diabetes mellitus and hypertension, and was treated with metformin, glipizide, hydrochlorothiazide, and losartan. Neurological examinations showed no neurological signs or symptoms above the neck. He ⇑ Tel.: +886 6 276 6187; fax: +886 6 237 4285.
had quadriparesis (muscle power grade 3-4, worse on the left side), hyporeflexia at bilateral C5-6 level, hyperreflexia in his bilateral triceps, spasticity and hyperreflexia in the lower limbs, bilateral extensor plantar response, a sensory level at T3, and severely impaired joint position and vibration senses in four limbs, worse in the lower limbs. His sphincters were normal. He had difficulty in postural maintenance and his left upper limb dropped slowly and fell away when he outstretched his arms. The fingers showed involuntary, slow, writhing movements that were worse on the left side. When he was asked to close his eyes, the pseudoathetoid movements in the fingers increased and his trunk swayed markedly (Video 1). He had difficulty in writing, handling chopsticks, dressing himself, doing up buttons, and firmly grasping things. When he was supported to walk, he continually looked at his legs and the floor. His steps were clumsy, wide-based, and irregular. His foot placement was erratic and he swayed his trunk. His left leg was raised much higher than necessary and was brought down forcibly on the floor (stamping) (Video 2). Laboratory investigations showed normal vitamin B12 level, a negative syphilis serology test, and an abnormally high hemoglobin A1c level (8.1%). Cervical magnetic resonance imaging showed cervical spondylotic changes with disc extrusion and multilevel spinal cord compression from C3 to C7, which was most severe at C3-4 and C4-5 (Fig. 1). Somatosensory evoked potentials (SSEPs) showed normal latency at the Erb point and no pickup of high cervical and cortical responses with bilateral median and tibial nerve stimulations. He underwent C3-4 and C4-5 anterior discectomies
E-mail address: [email protected] http://dx.doi.org/10.1016/j.jocn.2016.08.004 0967-5868/Ó 2016 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hwang W-J. Reversible pseudoathetosis and sensory ataxic gait caused by cervical spondylotic myelopathy. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.08.004
2
Case Report / Journal of Clinical Neuroscience xxx (2016) xxx–xxx
The absence of dysarthria, nystagmus, and abnormal eye movements, and the presence of abnormal joint position senses and a positive Romberg sign suggest that the patient’s gait disorder was a sensory ataxic gait. Again, the ataxic gait occurred in the limbs with proprioceptive deficit and resolved when the sensory impairment had been ameliorated. These features further support that the ataxic gait in this case was due to a proprioceptive sensory deficit. Proprioceptive deafferentation has been reported to cause poor motor control of the affected limbs [1]. A firm constant grasp cannot be sustained because of pseudoathetoid movement of the twisting fingers. This patient showed clumsiness and misreaching on several motor tasks, especially without visual guidance. Opening the eyes may not necessarily improve the symptoms related to a proprioceptive deficit [2–4]. However, closing the eyes exacerbated the pseudoathetosis of the fingers in this case, as it does in others [1,3]. 4. Conclusion
Fig. 1. Preoperative T2-weighted MRI image showing cervical spondylotic changes with disc extrusion and multilevel spinal cord compression from C3 to C7, which was most severe at C3-4 and C4-5.
Cervical spondylosis is the most common extrinsic compressive lesion of the spinal cord. This case illustrates that a proprioceptive sensory deficit originating in the cervical cord can cause both pseudoathetosis in the patient’s deafferented fingers and sensory ataxic gait. This case highlights the clinical importance of recognizing a surgically treatable and reversible condition of the spinal cord that causes a great negative effect on the patient’s mobility and activities of daily living.
and anterior fusion. Two months postoperatively, the pseudoathetosis of the fingers was still present; however, the postural maintenance of his outstretched arms and his gait had clearly improved. He sometimes used a cane as a mobility aid (Videos 3 and 4). About 7 months postoperatively, the patient’s pseudoathetosis of the fingers was mild but exacerbated when he closed his eyes. He could walk steadily without any mobility aid. Turning did not induce significant postural perturbation (Video 5). The joint position sense of his four limbs markedly improved (one mistake in 10 trials). A follow-up SSEPs study showed normal Erb latency, mildly prolonged Erb-C2 latency, and normal C2-cortical latency with bilateral median nerve stimulation. The cortical responses were mildly prolonged with bilateral tibial nerve stimulation.
Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jocn.2016.08.004.
3. Discussion
References
Spinal pseudoathetosis has been reported in association with various causes, including demyelination [2], inflammation [2], syringomyelia [3], and cervical disc extrusion [4,5]. In this case, the movement disorders occurred at the body parts associated with a proprioceptive deficit, and the abnormal movements resolved when the sensory loss improved (Video 5). These features suggest pseudoathetosis associated with a proprioceptive deficit rather than athetosis of striatal origin.
Conflicts of Interest/Financial Disclosures Nothing to report. Funding, sponsorship No corporate, government, or institutional funding. Appendix A. Supplementary data
[1] Rothwell JC, Traub MM, Day BL, et al. Manual motor performance in a deafferented man. Brain 1982;105:515–42. [2] Sharp FR, Rando TA, Greenberg SA, et al. Pseudochoreoathetosis. Movements associated with loss of proprioception. Arch Neurol 1994;51:1103–9. [3] Spitz M, Costa Machado AA, Carvalho Rdo C, et al. Pseudoathetosis: report of three patients. Mov Disord 2006;21:1520–2. [4] Bray K, Chhetri SK, Varma A, et al. Reversible pseudoathetosis induced by cervical myelopathy. Mov Disord 2012;27:1370–1. [5] Nakajima M, Hirayama K. Midcervical central cord syndrome: numb and clumsy hands due to midline cervical disc protrusion at the C3-4 intervertebral level. J Neurol Neurosurg Psychiatry 1995;58:607–13.
Please cite this article in press as: Hwang W-J. Reversible pseudoathetosis and sensory ataxic gait caused by cervical spondylotic myelopathy. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.08.004
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Reversible pseudoathetosis and sensory ataxic gait caused by cervical spondylotic myelopathy Wen-Juh Hwang ⇑ Department of Neurology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, 138 Sheng-Li Road, Tainan 704, Taiwan
a r t i c l e
i n f o
Article history: Received 5 June 2016 Accepted 1 August 2016 Available online xxxx Keywords: Cervical spondylotic myelopathy Pseudoathetosis Sensory ataxic gait
a b s t r a c t Proprioceptive deafferentation of spinal cord origin can cause pseudoathetosis, sensory ataxic gait, or both. The co-existence of pseudoathetosis and sensory ataxic gait caused by a surgically treatable condition of the spinal cord has been rarely reported. An 80-year-old man with cervical spondylotic myelopathy presented with severe sensory ataxic gait which confined him to a wheelchair. He also had poor control of his hands due to the pseudoathetoid movements of the fingers, which prevented him from sustaining constant muscle contraction. He underwent C3-4 and C4-5 anterior discectomies and anterior fusion. His neurological deficits gradually improved after the decompressive surgery. About 7 months postoperatively, he was totally independent in activities of daily living and needed no mobility aid. This case highlights the clinical importance of recognizing a surgically treatable and reversible condition of the spinal cord that causes pseudoathetosis and sensory ataxic gait. Ó 2016 Elsevier Ltd. All rights reserved.
1. Introduction Athetosis refers to an involuntary, slow, writhing movement primarily affecting the distal parts of the extremities. Pseudoathetosis is clinically indistinguishable from athetosis and is caused by impaired proprioception. Pseudoathetosis can occur with lesions located anywhere along the proprioceptive sensory pathways from the peripheral nerves to the parietal cortex [1–5]. While the exact mechanism of pseudoathetosis is uncertain, it is hypothesized that it occurs because of the failure of the striatum to properly integrate cortical motor and sensory inputs [2]. I report a patient with cervical spondylotic myelopathy with severe deafferentation of the four limbs that manifested as sensory ataxic gait and pseudoathetosis in the fingers. His neurological deficits markedly improved after surgical decompression. 2. Case report An 80-year-old man presented with poor control of his hands, paresthesia of the four limbs, and progressive unsteady gait for the previous 6 months. He had a medical history of diabetes mellitus and hypertension, and was treated with metformin, glipizide, hydrochlorothiazide, and losartan. Neurological examinations showed no neurological signs or symptoms above the neck. He ⇑ Tel.: +886 6 276 6187; fax: +886 6 237 4285.
had quadriparesis (muscle power grade 3-4, worse on the left side), hyporeflexia at bilateral C5-6 level, hyperreflexia in his bilateral triceps, spasticity and hyperreflexia in the lower limbs, bilateral extensor plantar response, a sensory level at T3, and severely impaired joint position and vibration senses in four limbs, worse in the lower limbs. His sphincters were normal. He had difficulty in postural maintenance and his left upper limb dropped slowly and fell away when he outstretched his arms. The fingers showed involuntary, slow, writhing movements that were worse on the left side. When he was asked to close his eyes, the pseudoathetoid movements in the fingers increased and his trunk swayed markedly (Video 1). He had difficulty in writing, handling chopsticks, dressing himself, doing up buttons, and firmly grasping things. When he was supported to walk, he continually looked at his legs and the floor. His steps were clumsy, wide-based, and irregular. His foot placement was erratic and he swayed his trunk. His left leg was raised much higher than necessary and was brought down forcibly on the floor (stamping) (Video 2). Laboratory investigations showed normal vitamin B12 level, a negative syphilis serology test, and an abnormally high hemoglobin A1c level (8.1%). Cervical magnetic resonance imaging showed cervical spondylotic changes with disc extrusion and multilevel spinal cord compression from C3 to C7, which was most severe at C3-4 and C4-5 (Fig. 1). Somatosensory evoked potentials (SSEPs) showed normal latency at the Erb point and no pickup of high cervical and cortical responses with bilateral median and tibial nerve stimulations. He underwent C3-4 and C4-5 anterior discectomies
E-mail address: [email protected] http://dx.doi.org/10.1016/j.jocn.2016.08.004 0967-5868/Ó 2016 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hwang W-J. Reversible pseudoathetosis and sensory ataxic gait caused by cervical spondylotic myelopathy. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.08.004
2
Case Report / Journal of Clinical Neuroscience xxx (2016) xxx–xxx
The absence of dysarthria, nystagmus, and abnormal eye movements, and the presence of abnormal joint position senses and a positive Romberg sign suggest that the patient’s gait disorder was a sensory ataxic gait. Again, the ataxic gait occurred in the limbs with proprioceptive deficit and resolved when the sensory impairment had been ameliorated. These features further support that the ataxic gait in this case was due to a proprioceptive sensory deficit. Proprioceptive deafferentation has been reported to cause poor motor control of the affected limbs [1]. A firm constant grasp cannot be sustained because of pseudoathetoid movement of the twisting fingers. This patient showed clumsiness and misreaching on several motor tasks, especially without visual guidance. Opening the eyes may not necessarily improve the symptoms related to a proprioceptive deficit [2–4]. However, closing the eyes exacerbated the pseudoathetosis of the fingers in this case, as it does in others [1,3]. 4. Conclusion
Fig. 1. Preoperative T2-weighted MRI image showing cervical spondylotic changes with disc extrusion and multilevel spinal cord compression from C3 to C7, which was most severe at C3-4 and C4-5.
Cervical spondylosis is the most common extrinsic compressive lesion of the spinal cord. This case illustrates that a proprioceptive sensory deficit originating in the cervical cord can cause both pseudoathetosis in the patient’s deafferented fingers and sensory ataxic gait. This case highlights the clinical importance of recognizing a surgically treatable and reversible condition of the spinal cord that causes a great negative effect on the patient’s mobility and activities of daily living.
and anterior fusion. Two months postoperatively, the pseudoathetosis of the fingers was still present; however, the postural maintenance of his outstretched arms and his gait had clearly improved. He sometimes used a cane as a mobility aid (Videos 3 and 4). About 7 months postoperatively, the patient’s pseudoathetosis of the fingers was mild but exacerbated when he closed his eyes. He could walk steadily without any mobility aid. Turning did not induce significant postural perturbation (Video 5). The joint position sense of his four limbs markedly improved (one mistake in 10 trials). A follow-up SSEPs study showed normal Erb latency, mildly prolonged Erb-C2 latency, and normal C2-cortical latency with bilateral median nerve stimulation. The cortical responses were mildly prolonged with bilateral tibial nerve stimulation.
Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jocn.2016.08.004.
3. Discussion
References
Spinal pseudoathetosis has been reported in association with various causes, including demyelination [2], inflammation [2], syringomyelia [3], and cervical disc extrusion [4,5]. In this case, the movement disorders occurred at the body parts associated with a proprioceptive deficit, and the abnormal movements resolved when the sensory loss improved (Video 5). These features suggest pseudoathetosis associated with a proprioceptive deficit rather than athetosis of striatal origin.
Conflicts of Interest/Financial Disclosures Nothing to report. Funding, sponsorship No corporate, government, or institutional funding. Appendix A. Supplementary data
[1] Rothwell JC, Traub MM, Day BL, et al. Manual motor performance in a deafferented man. Brain 1982;105:515–42. [2] Sharp FR, Rando TA, Greenberg SA, et al. Pseudochoreoathetosis. Movements associated with loss of proprioception. Arch Neurol 1994;51:1103–9. [3] Spitz M, Costa Machado AA, Carvalho Rdo C, et al. Pseudoathetosis: report of three patients. Mov Disord 2006;21:1520–2. [4] Bray K, Chhetri SK, Varma A, et al. Reversible pseudoathetosis induced by cervical myelopathy. Mov Disord 2012;27:1370–1. [5] Nakajima M, Hirayama K. Midcervical central cord syndrome: numb and clumsy hands due to midline cervical disc protrusion at the C3-4 intervertebral level. J Neurol Neurosurg Psychiatry 1995;58:607–13.
Please cite this article in press as: Hwang W-J. Reversible pseudoathetosis and sensory ataxic gait caused by cervical spondylotic myelopathy. J Clin Neurosci (2016), http://dx.doi.org/10.1016/j.jocn.2016.08.004