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Paroxysmal alternating nystagmus
Journal of the Neurological Sciences 318 (2012) 181–184
Contents lists available at SciVerse ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Paroxysmal alternating nystagmus☆ Seong-Joon Lee a, Sung-Yeon Sohn a, Ji Soo Kim b, So Young Moon a,⁎ a b
Department of Neurology, Ajou University School of Medicine, Suwon, Republic of Korea Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Gyeonggi-do, Republic of Korea
a r t i c l e
i n f o
Article history: Received 5 December 2011 Received in revised form 15 March 2012 Accepted 15 March 2012 Available online 1 May 2012 Keywords: Paroxysmal alternating nystagmus Vertigo Vestibulopathy Medulloblastoma Carbamazepine
a b s t r a c t Paroxysmal reversal of spontaneous nystagmus has rarely been described. Herein, we report recurrent bouts of vertigo and oscillopsia in association with reversed spontaneous nystagmus in a patient with prior surgical resection of the cerebellar vermis due to medulloblastoma and subsequent labyrinthine damage. The symptoms almost completely resolved with carbamazepine. The episodes may be ascribed to paroxysmal reversal of the vestibular asymmetry due to intermittent decompensation by the deficient cerebellum. © 2012 Elsevier B.V. All rights reserved.
1. Introduction Reversal of spontaneous nystagmus may occur in recovery nystagmus [1], periodic or aperiodic alternating nystagmus [2], and epileptic nystagmus [3]. However, paroxysmal reversal of spontaneous nystagmus has rarely been described. Herein, we report recurrent bouts of vertigo and oscillopsia in association with paroxysmal reversal of the spontaneous nystagmus in a patient with a prior surgical resection of the cerebellar medulloblastoma and subsequent labyrinthine damage. The paroxysmal reversal of spontaneous nystagmus suggests intermittent decompensation of the vestibular asymmetry due to cerebellar dysfunction and resultant vestibular excitation. The symptoms almost completely resolved with carbamazepine. 2. Case report A 20 year old man presented with paroxysmal attacks of oscillopsia and vertigo for 3 months. In August 2000, he underwent a gross total resection of fourth ventricle medulloblastoma with leptomeningeal seeding. He also had received post-operative radio- and chemotherapy over the following 2 years, and was declared to be completely cured. In March 2004, he experienced sudden hearing loss on the left side. At that time, he had 80 dB sensorineural hearing loss on pure ☆ This study was supported by a grant of the Korea Health 21 R&D Project, Ministry of Health, Welfare, and Family Affairs, Republic of Korea (A102065). ⁎ Corresponding author at: Department of Neurology, School of Medicine, Ajou University, San 5, Woncheon-dong, Yeongtong-gu, Suwon, Kyungki-do, 443-721, Republic of Korea. Tel.: + 82 31 219 5175; fax: + 82 31 219 5178. E-mail address: [email protected] (S.Y. Moon). 0022-510X/$ – see front matter © 2012 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2012.03.010
tone audiometry and normal caloric responses. Since then, he had suffered from continuous left side hearing loss and tinnitus. In November 2009, while eating hot food, he suddenly experienced vertigo for 30 s. The attacks became more frequent, but spinal and brain MRIs did not show any evidence of tumor recurrence. Medication of diuretics and flunarizine was in vain, and the attacks increased in frequency to more than 100 episodes a day. Between the attacks, the patient showed right beating horizontal–torsional spontaneous nystagmus with upbeat components (Fig. 1A and Video). The spontaneous nystagmus increased in darkness. Head impulse test (HIT) was positive leftward. He had normal eye alignment and a full range of eye movements. Smooth pursuit was normal, with suspicious hypermetric rightward saccades. Provocation maneuvers such as head shaking or vibration stimuli on the mastoids were unrevealing. The nystagmus did not change during positioning/positional maneuvers. He was free of any motor, sensory, and cerebellar signs. In contrast, when he developed vertigo and oscillopsia, the right beating horizontal–torsional spontaneous nystagmus changed into vigorous left beating horizontal–torsional nystagmus (Fig. 1B and Video). The nystagmus showed a crescendo–decrescendo pattern over 60 to 70 s and suppression with visual fixation. During the attacks, the patient tilted his head leftward, and HIT was positive rightward. With completion of the attack, the right beating nystagmus resumed with a brief increase, and then returned to the baseline status. Evaluation showed 80 dB sensorineural hearing loss in the left ear and no wave formation on brainstem auditory evoked potential during left ear stimulation, which is suggestive of left cochlear or acoustic nerve lesion. He also showed complete left caloric paralysis. EEG and CSF examination were normal without oligoclonal bands or malignant cells. Brain MRI confirmed a previous removal of the uvula,
182
S.-J. Lee et al. / Journal of the Neurological Sciences 318 (2012) 181–184
Fig. 1. Between the attacks, our patient shows continuous spontaneous right beating horizontal–torsional nystagmus with upbeat component. During the attacks, the nystagmus changes into large amplitude left beating horizontal–torsional nystagmus. The ictal nystagmus has a crescendo–decrescendo pattern over 30 to 40 s. The nystagmus returns to the interictal pattern without a null period. Please note temporary increase of the interictal nystagmus after the paroxysm. LH, left horizontal; LV, left vertical; LT, left torsional.
pyramid, nodulus, and bilateral biventral lobule without a new lesion (Fig. 2). Baclofen up to 80 mg a day was ineffective, but carbamazepine 200 mg twice a day brought a near complete resolution of the episodes. 3. Discussion Our patient developed bouts of vertigo and oscillopsia along with paroxysmal reversal of the spontaneous nystagmus. The periodic reversal of spontaneous nystagmus is a typical feature of periodic alternating nystagmus (PAN). However, the characteristics of nystagmus reversal observed in our patient are distinct from those found in PAN. First, the duration and amplitude of each nystagmus were markedly asymmetric in our patient. Second, our patient lacks the classic null period described in PAN. Third, while PAN has a regular periodicity, our patient's episodes of direction changing nystagmus were unpredictable. Fourth, while PAN usually does not cause oscillopsia and paroxysmal vertigo, our patient experienced both during the attacks. Last, while baclofen is known to be effective in PAN, our patient responded to carbamazepine instead of baclofen. Recovery nystagmus is a direction changing nystagmus that is commonly seen following cessation of prolonged unidirectional vestibular stimulation, or at the recovery phase of unilateral vestibular damage. It is explained by adaptive mechanism and is commonly seen in Ménière's disease [4,5]. This also differs from the feature observed in our patient in that the episodes were much more frequent and the durations were brief. There has been a report on a patient with epileptic periodic alternating nystagmus who also showed periodic alternating gaze deviation and head rotation as a manifestation of a seizure after awakening from hypoxic ischemic encephalopathy [3]. These features are obviously different from those in our patient. In our patient, in view of the relatively weak nystagmus between the attacks and canal paralysis, the interictal positive HIT may be ascribed to canal paresis. However, the positive HIT in the opposite direction during the paroxysms may be a manifestation of severe asymmetry of vestibule-ocular reflex due to directional preponderance. In view of the combined audiovestibular loss and no interval changes in the brain MRI, the lesion appears to be located in the labyrinth. However, the cause of the lesion remains speculative. The long time interval between the hearing loss and vestibulopathy suggest unrelated pathologies or delayed hydrops. Paroxysmal or ‘aperiodic’
Fig. 2. MRIs show an absence of the inferior cerebellar vermis and bilateral biventral lobules due to prior surgical resection.
alternating nystagmus has been described only in a few patients (Table 1). Among them, a woman with suspected brain stem glioma involving the area of left vestibular nuclei and cerebellar peduncle developed paroxysmal alternating skew deviation and direction changing nystagmus after a biopsy in the inferior cerebellar vermis and resultant destruction of the uvula. Between the attacks, she had right over left skew deviation with asymptomatic right beating horizontal nystagmus. Slow phases of the nystagmus were of increasing velocity, similar to congenital nystagmus. Every 40 to 50 s, paroxysmal reversal of her skew deviation accompanied severe oscillopsia and vigorous left beating horizontal torsional nystagmus for 10–12 s [6]. This case and our patient share the underlying lesions in the cerebellar vermis and accompanying unilateral or asymmetric vestibulopathy. The underlying mechanism of PAN is known as relative instability in the velocity storage element, and it may represent the activity of a biological oscillator. PAN has been ascribed to lesions involving the cerebellar uvula and nodulus or their connections with the brainstem vestibular nuclei [7]. Destruction of the uvula and nodulus was apparent in our patient and the patient described previously. However, unlike in classic PAN, intermittent decompensation by the already deficient cerebellum may have caused paroxysmal reversal of the vestibular activity in our patient with underlying unilateral vestibular dysfunction. The nystagmus in our patient responded to carbamazepine and not to baclofen. Baclofen is known to be effective in PAN, possibly by mimicking the inhibitory GABAergic actions of the nodular Purkinje cells over the vestibular nuclei [8]. In contrast, carbamazepine is mostly effective in the disorders characterized by paroxysmal neural irritation, such as vestibular paroxysmia, in which ephaptic spreading of action potentials is known to cause abnormal vestibular excitation [9]. The response to carbamazepine in our patient favors an intermittent vestibular excitation as a mechanism of the paroxysms in our patient. Previously, a 55-year-old woman developed paroxysms of vertigo and visual blurring in association with combined torsional–horizontal–vertical nystagmus occurring every 2 min and lasting for 15 s. Between the attacks, there was a much finer
S.-J. Lee et al. / Journal of the Neurological Sciences 318 (2012) 181–184
183
Table 1 Previous reports of Paroxysmal or ‘aperiodic’ alternating nystagmus. Patient
1986, Nuti et al. case 1, M/ 68 [11]
1986, Nuti et al. Case 2, M/ 50 [11]
1995, Lawden et al. F/55 [10]
2001, Radtke et al. F/29 [6]
2011, Pfeffer et al. M/55 [12]
Our case
Chief complaint
Oscillopsia, instability
Vertigo, headache, oscillopsia Horizontal rotatory left beating nystagmus (40–60 s) Nonperiodic Rt beating phases(b 20 s),
Episodic Vertigo, oscillopsia
Episodic vertigo, oscillopsia Spontaneous right horizontal nystagmus
Oscillopsia
Episodic vertigo, oscillopsia
Subtle left beating nystagmus
Right beating spontaneous nystagmus with clockwise torsional and upbeat components
Left beating nystagmus (10–15 s)
Intermittent right beating horizontal nystagmus (5–15 s)
Vigorous left beating nystagmus with counterclockwise torsional component (60–70 s)
40–50 s NONE None
Nonperiodic None None
None
Spontaneous Spontaneous nystagmus left beating
Rare jerks of Direction right beating changing nystagmus nystagmus lasting 2 ~ 15 s Interval Null point Gaze evoked nystagmus Headshaking nystagmus Positioning
Nonperiodic None None
None
None
Saccade
Leftward saccadic overshoot
Smooth pursuit
Inability to perform smooth pursuit Bilateral Vestibular hyperreflexia
Vestibular function
Nonperiodic None Positive, with rebound nystagmus None
Fine asymptomatic contralateral beating nystagmus, Regular 2 minute interval torsional, horizontal, and vertical nystagmus,(15 s) Regular 2 minute None None
None
None
Irregular None Horizontal with rebound nystagmus Not described
None
None
Not described
Normal
Normal
Normal
Normal
Saccadic to left
Right hypermetric saccades Dysmetric, with occasional macrosaccadic oscillations Not described Normal
Bilateral vestibular hyperreflexia
Left caloric paresis
Left caloric paresis
Central positional nystagmus Leftward saccadic undershoot
Other points No brain MRI No brain MRI of note
Alternating skew deviation
Etiology
Left vertebral 4 months after cervical artery whiplash stenosis injury due to car accident
Arteriovenous malformation near left vestibular nucleus with previous bleeding
Treatment
Baclofen
Carbamazepine
Not tried
Underlying brainstem glioma near left vestibular nuclei and cerebellar peduncle, with biopsy damage to uvula Baclofen
None
Lt caloric paresis Impaired pursuit and cancelation of vestibuleocular reflex Onset after 1 year of initial presentation, after using steroids and antibiotics Medullary and Previous surgical removal of uvula, pyramid, nodulus, and bilateral biventral lobule, with previous history of Left sensorineural hearing los cervical cord atrophy due to Adult-onset Alexander disease
Spontaneous improvement
nystagmus in the opposite direction of the nystagmus during the paroxysms. MRI revealed an arteriovenous malformation in close proximity to the left vestibular nucleus, with an evidence of previous bleeding. Her symptoms and the nystagmus also responded to carbamazepine [10]. In summary, the paroxysmal reversal of spontaneous nystagmus suggests intermittent decompensation of the vestibular asymmetry due to cerebellar dysfunction and resultant vestibular excitation. Supplementary related to this article can be found online at doi:10.1016/j.jns.2012.03.010.
Carbamazepine, unresponsive to baclofen
Conflict of interest None.
References [1] Jacobson GP, Pearlstein R, Henderson J, Calder JH, Rock J. Recovery nystagmus revisited. J Am Acad Audiol 1998;9:263–71. [2] Baloh RW, Honrubia V, Konrad HR. Periodic alternating nystagmus. Brain J Neurol 1976;99:11–26.
184
S.-J. Lee et al. / Journal of the Neurological Sciences 318 (2012) 181–184
[3] Moster ML, Schnayder E. Epileptic periodic alternating nystagmus. J NeuroOphthalmol Off J N Am Neuro-Ophthalmol Soc 1998;18:292–3. [4] McClure JA, Lycett P. Recovery nystagmus. J Otolaryngol 1978;7:141–8. [5] McClure JA, Copp JC, Lycett P. Recovery nystagmus in Meniere's disease. Laryngoscope 1981;91:1727–37. [6] Radtke A, Bronstein AM, Gresty MA, Faldon M, Taylor W, Stevens JM, et al. Paroxysmal alternating skew deviation and nystagmus after partial destruction of the uvula. J Neurol Neurosurg Psychiatry 2001;70:790–3. [7] Furman JM, Wall III C, Pang DL. Vestibular function in periodic alternating nystagmus. Brain J Neurol 1990;113(Pt 5):1425–39. [8] Isago H, Tsuboya R, Kataura A. A case of periodic alternating nystagmus: with a special reference to the efficacy of baclofen treatment. Auris Nasus Larynx 1985;12: 15–21.
[9] Hufner K, Barresi D, Glaser M, Linn J, Adrion C, Mansmann U, et al. Vestibular paroxysmia: diagnostic features and medical treatment. Neurology 2008;71:1006–14. [10] Lawden MC, Bronstein AM, Kennard C. Repetitive paroxysmal nystagmus and vertigo. Neurology 1995;45:276–80. [11] Nuti D, Ciacci G, Giannini F, Rossi A, Federico A. Aperiodic alternating nystagmus: report of two cases and treatment by baclofen. Ital J Neurol Sci 1986;7:453–9. [12] Pfeffer G, Abegg M, Vertinsky AT, Ceccherini I, Caroli F, Barton JJ. The ocular motor features of adult-onset Alexander disease: a case and review of the literature. J Neuro-Ophthalmol Off J N Am Neuro-Ophthalmol Soc 2011;31:155–9.
Contents lists available at SciVerse ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Short communication
Paroxysmal alternating nystagmus☆ Seong-Joon Lee a, Sung-Yeon Sohn a, Ji Soo Kim b, So Young Moon a,⁎ a b
Department of Neurology, Ajou University School of Medicine, Suwon, Republic of Korea Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Gyeonggi-do, Republic of Korea
a r t i c l e
i n f o
Article history: Received 5 December 2011 Received in revised form 15 March 2012 Accepted 15 March 2012 Available online 1 May 2012 Keywords: Paroxysmal alternating nystagmus Vertigo Vestibulopathy Medulloblastoma Carbamazepine
a b s t r a c t Paroxysmal reversal of spontaneous nystagmus has rarely been described. Herein, we report recurrent bouts of vertigo and oscillopsia in association with reversed spontaneous nystagmus in a patient with prior surgical resection of the cerebellar vermis due to medulloblastoma and subsequent labyrinthine damage. The symptoms almost completely resolved with carbamazepine. The episodes may be ascribed to paroxysmal reversal of the vestibular asymmetry due to intermittent decompensation by the deficient cerebellum. © 2012 Elsevier B.V. All rights reserved.
1. Introduction Reversal of spontaneous nystagmus may occur in recovery nystagmus [1], periodic or aperiodic alternating nystagmus [2], and epileptic nystagmus [3]. However, paroxysmal reversal of spontaneous nystagmus has rarely been described. Herein, we report recurrent bouts of vertigo and oscillopsia in association with paroxysmal reversal of the spontaneous nystagmus in a patient with a prior surgical resection of the cerebellar medulloblastoma and subsequent labyrinthine damage. The paroxysmal reversal of spontaneous nystagmus suggests intermittent decompensation of the vestibular asymmetry due to cerebellar dysfunction and resultant vestibular excitation. The symptoms almost completely resolved with carbamazepine. 2. Case report A 20 year old man presented with paroxysmal attacks of oscillopsia and vertigo for 3 months. In August 2000, he underwent a gross total resection of fourth ventricle medulloblastoma with leptomeningeal seeding. He also had received post-operative radio- and chemotherapy over the following 2 years, and was declared to be completely cured. In March 2004, he experienced sudden hearing loss on the left side. At that time, he had 80 dB sensorineural hearing loss on pure ☆ This study was supported by a grant of the Korea Health 21 R&D Project, Ministry of Health, Welfare, and Family Affairs, Republic of Korea (A102065). ⁎ Corresponding author at: Department of Neurology, School of Medicine, Ajou University, San 5, Woncheon-dong, Yeongtong-gu, Suwon, Kyungki-do, 443-721, Republic of Korea. Tel.: + 82 31 219 5175; fax: + 82 31 219 5178. E-mail address: [email protected] (S.Y. Moon). 0022-510X/$ – see front matter © 2012 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2012.03.010
tone audiometry and normal caloric responses. Since then, he had suffered from continuous left side hearing loss and tinnitus. In November 2009, while eating hot food, he suddenly experienced vertigo for 30 s. The attacks became more frequent, but spinal and brain MRIs did not show any evidence of tumor recurrence. Medication of diuretics and flunarizine was in vain, and the attacks increased in frequency to more than 100 episodes a day. Between the attacks, the patient showed right beating horizontal–torsional spontaneous nystagmus with upbeat components (Fig. 1A and Video). The spontaneous nystagmus increased in darkness. Head impulse test (HIT) was positive leftward. He had normal eye alignment and a full range of eye movements. Smooth pursuit was normal, with suspicious hypermetric rightward saccades. Provocation maneuvers such as head shaking or vibration stimuli on the mastoids were unrevealing. The nystagmus did not change during positioning/positional maneuvers. He was free of any motor, sensory, and cerebellar signs. In contrast, when he developed vertigo and oscillopsia, the right beating horizontal–torsional spontaneous nystagmus changed into vigorous left beating horizontal–torsional nystagmus (Fig. 1B and Video). The nystagmus showed a crescendo–decrescendo pattern over 60 to 70 s and suppression with visual fixation. During the attacks, the patient tilted his head leftward, and HIT was positive rightward. With completion of the attack, the right beating nystagmus resumed with a brief increase, and then returned to the baseline status. Evaluation showed 80 dB sensorineural hearing loss in the left ear and no wave formation on brainstem auditory evoked potential during left ear stimulation, which is suggestive of left cochlear or acoustic nerve lesion. He also showed complete left caloric paralysis. EEG and CSF examination were normal without oligoclonal bands or malignant cells. Brain MRI confirmed a previous removal of the uvula,
182
S.-J. Lee et al. / Journal of the Neurological Sciences 318 (2012) 181–184
Fig. 1. Between the attacks, our patient shows continuous spontaneous right beating horizontal–torsional nystagmus with upbeat component. During the attacks, the nystagmus changes into large amplitude left beating horizontal–torsional nystagmus. The ictal nystagmus has a crescendo–decrescendo pattern over 30 to 40 s. The nystagmus returns to the interictal pattern without a null period. Please note temporary increase of the interictal nystagmus after the paroxysm. LH, left horizontal; LV, left vertical; LT, left torsional.
pyramid, nodulus, and bilateral biventral lobule without a new lesion (Fig. 2). Baclofen up to 80 mg a day was ineffective, but carbamazepine 200 mg twice a day brought a near complete resolution of the episodes. 3. Discussion Our patient developed bouts of vertigo and oscillopsia along with paroxysmal reversal of the spontaneous nystagmus. The periodic reversal of spontaneous nystagmus is a typical feature of periodic alternating nystagmus (PAN). However, the characteristics of nystagmus reversal observed in our patient are distinct from those found in PAN. First, the duration and amplitude of each nystagmus were markedly asymmetric in our patient. Second, our patient lacks the classic null period described in PAN. Third, while PAN has a regular periodicity, our patient's episodes of direction changing nystagmus were unpredictable. Fourth, while PAN usually does not cause oscillopsia and paroxysmal vertigo, our patient experienced both during the attacks. Last, while baclofen is known to be effective in PAN, our patient responded to carbamazepine instead of baclofen. Recovery nystagmus is a direction changing nystagmus that is commonly seen following cessation of prolonged unidirectional vestibular stimulation, or at the recovery phase of unilateral vestibular damage. It is explained by adaptive mechanism and is commonly seen in Ménière's disease [4,5]. This also differs from the feature observed in our patient in that the episodes were much more frequent and the durations were brief. There has been a report on a patient with epileptic periodic alternating nystagmus who also showed periodic alternating gaze deviation and head rotation as a manifestation of a seizure after awakening from hypoxic ischemic encephalopathy [3]. These features are obviously different from those in our patient. In our patient, in view of the relatively weak nystagmus between the attacks and canal paralysis, the interictal positive HIT may be ascribed to canal paresis. However, the positive HIT in the opposite direction during the paroxysms may be a manifestation of severe asymmetry of vestibule-ocular reflex due to directional preponderance. In view of the combined audiovestibular loss and no interval changes in the brain MRI, the lesion appears to be located in the labyrinth. However, the cause of the lesion remains speculative. The long time interval between the hearing loss and vestibulopathy suggest unrelated pathologies or delayed hydrops. Paroxysmal or ‘aperiodic’
Fig. 2. MRIs show an absence of the inferior cerebellar vermis and bilateral biventral lobules due to prior surgical resection.
alternating nystagmus has been described only in a few patients (Table 1). Among them, a woman with suspected brain stem glioma involving the area of left vestibular nuclei and cerebellar peduncle developed paroxysmal alternating skew deviation and direction changing nystagmus after a biopsy in the inferior cerebellar vermis and resultant destruction of the uvula. Between the attacks, she had right over left skew deviation with asymptomatic right beating horizontal nystagmus. Slow phases of the nystagmus were of increasing velocity, similar to congenital nystagmus. Every 40 to 50 s, paroxysmal reversal of her skew deviation accompanied severe oscillopsia and vigorous left beating horizontal torsional nystagmus for 10–12 s [6]. This case and our patient share the underlying lesions in the cerebellar vermis and accompanying unilateral or asymmetric vestibulopathy. The underlying mechanism of PAN is known as relative instability in the velocity storage element, and it may represent the activity of a biological oscillator. PAN has been ascribed to lesions involving the cerebellar uvula and nodulus or their connections with the brainstem vestibular nuclei [7]. Destruction of the uvula and nodulus was apparent in our patient and the patient described previously. However, unlike in classic PAN, intermittent decompensation by the already deficient cerebellum may have caused paroxysmal reversal of the vestibular activity in our patient with underlying unilateral vestibular dysfunction. The nystagmus in our patient responded to carbamazepine and not to baclofen. Baclofen is known to be effective in PAN, possibly by mimicking the inhibitory GABAergic actions of the nodular Purkinje cells over the vestibular nuclei [8]. In contrast, carbamazepine is mostly effective in the disorders characterized by paroxysmal neural irritation, such as vestibular paroxysmia, in which ephaptic spreading of action potentials is known to cause abnormal vestibular excitation [9]. The response to carbamazepine in our patient favors an intermittent vestibular excitation as a mechanism of the paroxysms in our patient. Previously, a 55-year-old woman developed paroxysms of vertigo and visual blurring in association with combined torsional–horizontal–vertical nystagmus occurring every 2 min and lasting for 15 s. Between the attacks, there was a much finer
S.-J. Lee et al. / Journal of the Neurological Sciences 318 (2012) 181–184
183
Table 1 Previous reports of Paroxysmal or ‘aperiodic’ alternating nystagmus. Patient
1986, Nuti et al. case 1, M/ 68 [11]
1986, Nuti et al. Case 2, M/ 50 [11]
1995, Lawden et al. F/55 [10]
2001, Radtke et al. F/29 [6]
2011, Pfeffer et al. M/55 [12]
Our case
Chief complaint
Oscillopsia, instability
Vertigo, headache, oscillopsia Horizontal rotatory left beating nystagmus (40–60 s) Nonperiodic Rt beating phases(b 20 s),
Episodic Vertigo, oscillopsia
Episodic vertigo, oscillopsia Spontaneous right horizontal nystagmus
Oscillopsia
Episodic vertigo, oscillopsia
Subtle left beating nystagmus
Right beating spontaneous nystagmus with clockwise torsional and upbeat components
Left beating nystagmus (10–15 s)
Intermittent right beating horizontal nystagmus (5–15 s)
Vigorous left beating nystagmus with counterclockwise torsional component (60–70 s)
40–50 s NONE None
Nonperiodic None None
None
Spontaneous Spontaneous nystagmus left beating
Rare jerks of Direction right beating changing nystagmus nystagmus lasting 2 ~ 15 s Interval Null point Gaze evoked nystagmus Headshaking nystagmus Positioning
Nonperiodic None None
None
None
Saccade
Leftward saccadic overshoot
Smooth pursuit
Inability to perform smooth pursuit Bilateral Vestibular hyperreflexia
Vestibular function
Nonperiodic None Positive, with rebound nystagmus None
Fine asymptomatic contralateral beating nystagmus, Regular 2 minute interval torsional, horizontal, and vertical nystagmus,(15 s) Regular 2 minute None None
None
None
Irregular None Horizontal with rebound nystagmus Not described
None
None
Not described
Normal
Normal
Normal
Normal
Saccadic to left
Right hypermetric saccades Dysmetric, with occasional macrosaccadic oscillations Not described Normal
Bilateral vestibular hyperreflexia
Left caloric paresis
Left caloric paresis
Central positional nystagmus Leftward saccadic undershoot
Other points No brain MRI No brain MRI of note
Alternating skew deviation
Etiology
Left vertebral 4 months after cervical artery whiplash stenosis injury due to car accident
Arteriovenous malformation near left vestibular nucleus with previous bleeding
Treatment
Baclofen
Carbamazepine
Not tried
Underlying brainstem glioma near left vestibular nuclei and cerebellar peduncle, with biopsy damage to uvula Baclofen
None
Lt caloric paresis Impaired pursuit and cancelation of vestibuleocular reflex Onset after 1 year of initial presentation, after using steroids and antibiotics Medullary and Previous surgical removal of uvula, pyramid, nodulus, and bilateral biventral lobule, with previous history of Left sensorineural hearing los cervical cord atrophy due to Adult-onset Alexander disease
Spontaneous improvement
nystagmus in the opposite direction of the nystagmus during the paroxysms. MRI revealed an arteriovenous malformation in close proximity to the left vestibular nucleus, with an evidence of previous bleeding. Her symptoms and the nystagmus also responded to carbamazepine [10]. In summary, the paroxysmal reversal of spontaneous nystagmus suggests intermittent decompensation of the vestibular asymmetry due to cerebellar dysfunction and resultant vestibular excitation. Supplementary related to this article can be found online at doi:10.1016/j.jns.2012.03.010.
Carbamazepine, unresponsive to baclofen
Conflict of interest None.
References [1] Jacobson GP, Pearlstein R, Henderson J, Calder JH, Rock J. Recovery nystagmus revisited. J Am Acad Audiol 1998;9:263–71. [2] Baloh RW, Honrubia V, Konrad HR. Periodic alternating nystagmus. Brain J Neurol 1976;99:11–26.
184
S.-J. Lee et al. / Journal of the Neurological Sciences 318 (2012) 181–184
[3] Moster ML, Schnayder E. Epileptic periodic alternating nystagmus. J NeuroOphthalmol Off J N Am Neuro-Ophthalmol Soc 1998;18:292–3. [4] McClure JA, Lycett P. Recovery nystagmus. J Otolaryngol 1978;7:141–8. [5] McClure JA, Copp JC, Lycett P. Recovery nystagmus in Meniere's disease. Laryngoscope 1981;91:1727–37. [6] Radtke A, Bronstein AM, Gresty MA, Faldon M, Taylor W, Stevens JM, et al. Paroxysmal alternating skew deviation and nystagmus after partial destruction of the uvula. J Neurol Neurosurg Psychiatry 2001;70:790–3. [7] Furman JM, Wall III C, Pang DL. Vestibular function in periodic alternating nystagmus. Brain J Neurol 1990;113(Pt 5):1425–39. [8] Isago H, Tsuboya R, Kataura A. A case of periodic alternating nystagmus: with a special reference to the efficacy of baclofen treatment. Auris Nasus Larynx 1985;12: 15–21.
[9] Hufner K, Barresi D, Glaser M, Linn J, Adrion C, Mansmann U, et al. Vestibular paroxysmia: diagnostic features and medical treatment. Neurology 2008;71:1006–14. [10] Lawden MC, Bronstein AM, Kennard C. Repetitive paroxysmal nystagmus and vertigo. Neurology 1995;45:276–80. [11] Nuti D, Ciacci G, Giannini F, Rossi A, Federico A. Aperiodic alternating nystagmus: report of two cases and treatment by baclofen. Ital J Neurol Sci 1986;7:453–9. [12] Pfeffer G, Abegg M, Vertinsky AT, Ceccherini I, Caroli F, Barton JJ. The ocular motor features of adult-onset Alexander disease: a case and review of the literature. J Neuro-Ophthalmol Off J N Am Neuro-Ophthalmol Soc 2011;31:155–9.