Parkinsonism and Related Disorders 21 (2015) 325e326
Contents lists available at ScienceDirect
Parkinsonism and Related Disorders journal homepage: www.elsevier.com/locate/parkreldis
Letter to the Editor
Clinical categorization of psychogenic blepharospasm To the Editor Benign essential blepharospasm is characterized by involuntary, bilateral, synchronous and stereotyped contraction of the orbicularis oculi muscles, together with an inability to voluntarily suppress the spasms. Its onset is insidious; its progression, gradual, without spontaneous remissions, although it may improve a little during initial medical consultation; and it worsens during voluntary movement or visual effort. The diagnosis of blepharospasm is presently based on clinical grounds, and it may be difficult to distinguish from other causes of eyelid closure, such as eye-closing tics [1]. A psychogenic cause may be suspected when incongruity of eyelid closure with the expression of essential blepharospasm, or inconsistency over time, is found. The aims of this work were: 1) to investigate whether recently proposed categories of psychogenic torsion dystonia [2], which are a revision of earlier criteria proposed by Fahn and Williams [3], could be applied to psychogenic blepharospasm; and 2) to identify specific motor manifestations for this aetiology. The clinical findings of five women and three men with suspected psychogenic blepharospasm, aged 31e57 years (mean age at onset, 42.5 years), were reviewed; the patients were recruited among a population of 40 patients diagnosed with blepharospasm at our clinic (20%). Each proband had a normal neurological examination, cranial MR imaging, and blood caeruloplasmin and copper levels, which ruled out other underlying neurological conditions. The authors analyzed whether each patient could fit into one category of psychogenic dystonia (Table 1). No patient exhibited presence of stereotyped, bilateral and synchronous spasms of the orbicularis oculi muscles, therefore ruling out essential blepharospasm [1], or fulfilled diagnostic criteria for apraxia of eyelid opening (i.e., (i) transitory inability to initiate lid opening; (ii) no evidence of ongoing orbicularis oculi contraction, such as lowering of the brows beneath the superior orbital margins; (iii) vigorous frontalis contraction during periods of inability to raise eyelids; (iv) no oculomotor or ocular sympathetic dysfunction, or ocular myopathy) [4]. No patient displayed dystonia in other body regions, or sensory tricks that reverted eyelid spasms. Although several patients received psychoactive drugs, the time course of involuntary eyelid closure excluded a diagnosis of drug-induced blepharospasm. A detailed presentation of the clinical features of these patients may be found in the Supplementary data. The category “documented psychogenic dystonia” is applied when dystonic symptoms are persistently relieved by psychotherapy, suggestion or placebo [2,5], or when the patient is witnessed free of symptoms when supposedly unobserved [2]. In this series, patients 1e4 experienced sustained relief of blepharospasm following http://dx.doi.org/10.1016/j.parkreldis.2014.12.005 1353-8020/© 2014 Elsevier Ltd. All rights reserved.
periodic administration of subcutaneous saline solution, and patient 4 was witnessed free of eyelid closure while supposedly unobserved. For these reasons, all of them could be ascribed to this group. Supplementary clues that suggested a psychogenic origin in this group were: 1) blepharospasm of sudden onset in patient 1; 2) changes in pattern and side of predominant eye closure in patient 2; 3) a decrease of eyelid closure during visual effort and when performing arithmetic calculations aloud, in patient 2; and 4) an absolute lack of response to treatments efficacious in blepharospasm (botulinum neurotoxin), in patients 1 and 4 [2,5]. The category “clinically established psychogenic dystonia plus other features” refers to abnormal movements inconsistent over time (i.e., spontaneous amelioration or remission of abnormal movements), or incongruous with the expression of organic dystonia (i.e., variability in the performance of involuntary movements, amelioration of dystonia during effort, bizarre movements), associated with psychogenic neurological signs, multiple somatizations or obvious psychiatric disturbances [2]. In this series, incongruous signs with respect to those of benign essential blepharospasm (eyelid narrowing in the absence of orbicularis oculi spasm) were found in patients 5 and 6; bizarre abnormal movements (bilateral eye convergence spasm) in patient 6, while inconsistency over time and non-stereotyped eyelid spasms became evident in patient 7. A diagnosis of psychiatric disease (conversion disorder, schizophrenia and major depression, respectively) had previously been made in each case; therefore, every one of them could be included in this class. The third category, “clinically established psychogenic dystonia minus other features”, refers to patients with unequivocal clinical features incompatible with organic disease, with no features suggesting another underlying neurological or psychiatric problem [2]. Patient 8 demonstrated inconsistency over time (spontaneous resolution of eyelid closure), and incongruity with essential blepharospasm (isolated contraction of procerus and corrugator muscles) in the absence of overt psychogenic or neurological disease, therefore fitting into this group. The three categories defined above are considered as “clinically definite” psychogenic dystonia [2]. Although our study group is too small to generate definitive conclusions, it is suggested from our results that the first objective of this study has been met, in that Gupta and Lang's guidelines to categorize psychogenic torsion dystonia [2], proved adequate for the diagnosis of isolated psychogenic blepharospasm in each patient of this series. On the opposite, specific clinical manifestations could not be identified for psychogenic blepharospasm. However, one motor sign found in this series, incongruous with the expression of essential blepharospasm, should draw attention to alternative aetiologies. This sign was the contraction of corrugator and procerus
326
Letter to the Editor / Parkinsonism and Related Disorders 21 (2015) 325e326
Table 1 Categories of psychogenic torsion dystonia, as defined by Gupta and Lang [3]. - Documented psychogenic dystonia Remittance with psychotherapy, suggestion, physiotherapy, or placebo. Dystonia absent while supposedly unobserved - Clinically established psychogenic dystonia, plus other features Inconsistent over time/incongruent with clinical condition plus other manifestations: obvious psychiatric disease, multiple somatizations, other “false” neurological signs - Clinically established psychogenic dystonia, minus other features Unequivocal clinical features incompatible with organic disease, with no features suggesting another underlying neurological or psychiatric problem
muscles (which causes narrowing of eyelid fissure and frowning of the eyebrows), in the absence of spasm of the orbicularis oculi. It was found in psychogenic blepharospasm in our patients, and may also be triggered by ophthalmic disease. Prognosis was favorable in this series, attesting to inconsistency over time of psychogenic blepharospasm. Spontaneous remission took place in four cases (50%), while the remaining patients experienced prolonged symptomatic relief from administration of placebo. It is worth mentioning that every patient in this series displayed isolated eyelid closure; if additional psychogenic neurological signs had been present, a diagnosis of functional movement disorder would have been more straightforward. Besides, no sensory tricks were reported, which was a significant clue for excluding essential blepharospasm, as these are considered highly specific features of dystonia [1]. Further research is needed to confirm our conclusions, and to adequately characterize the clinical characteristics of psychogenic blepharospasm.
Appendix A. Supplementary data Supplementary data related to this article can be found at http:// dx.doi.org/10.1016/j.parkreldis.2014.12.005.
References [1] Defazio G, Hallett M, Jinnah HA, Berardelli A. Development and validation of a guideline for diagnosing blepharospasm. Neurology 2013;81:236e40. [2] Gupta A, Lang AE. Psychogenic movement disorders. Curr Opin Neurol 2009;22: 430e6. [3] Fahn S, Williams DT. Psychogenic dystonia. Adv Neurol 1988;50:431e55. [4] Lepore FE, Duvoisin RC. “Apraxia” of eyelid opening: an involuntary levator inhibition. Neurology 1985;35:423e7. [5] Fasano A, Valadas A, Bathia KB, Prashanth DM, Lang AE, Munhoz RP, et al. Psychogenic facial movement disorders: clinical features and associated conditions. Mov Disord 2012;27:1544e51.
Gazulla*, Sebastia n García-Rubio Jose Department of Neurology, Hospital Universitario Miguel Servet, Zaragoza, Spain Carlota Ruiz-Gazulla Faculty of Biotechnology, University of Zaragoza, Zaragoza, Spain Pedro Modrego Department of Neurology, Hospital Universitario Miguel Servet, Zaragoza, Spain *
Corresponding author. Luis Vives 6, esc dcha, 7 B, 50006 Zaragoza, Spain. Tel.: þ34 976563649. E-mail address: [email protected] (J. Gazulla). 11 September 2014
Contents lists available at ScienceDirect
Parkinsonism and Related Disorders journal homepage: www.elsevier.com/locate/parkreldis
Letter to the Editor
Clinical categorization of psychogenic blepharospasm To the Editor Benign essential blepharospasm is characterized by involuntary, bilateral, synchronous and stereotyped contraction of the orbicularis oculi muscles, together with an inability to voluntarily suppress the spasms. Its onset is insidious; its progression, gradual, without spontaneous remissions, although it may improve a little during initial medical consultation; and it worsens during voluntary movement or visual effort. The diagnosis of blepharospasm is presently based on clinical grounds, and it may be difficult to distinguish from other causes of eyelid closure, such as eye-closing tics [1]. A psychogenic cause may be suspected when incongruity of eyelid closure with the expression of essential blepharospasm, or inconsistency over time, is found. The aims of this work were: 1) to investigate whether recently proposed categories of psychogenic torsion dystonia [2], which are a revision of earlier criteria proposed by Fahn and Williams [3], could be applied to psychogenic blepharospasm; and 2) to identify specific motor manifestations for this aetiology. The clinical findings of five women and three men with suspected psychogenic blepharospasm, aged 31e57 years (mean age at onset, 42.5 years), were reviewed; the patients were recruited among a population of 40 patients diagnosed with blepharospasm at our clinic (20%). Each proband had a normal neurological examination, cranial MR imaging, and blood caeruloplasmin and copper levels, which ruled out other underlying neurological conditions. The authors analyzed whether each patient could fit into one category of psychogenic dystonia (Table 1). No patient exhibited presence of stereotyped, bilateral and synchronous spasms of the orbicularis oculi muscles, therefore ruling out essential blepharospasm [1], or fulfilled diagnostic criteria for apraxia of eyelid opening (i.e., (i) transitory inability to initiate lid opening; (ii) no evidence of ongoing orbicularis oculi contraction, such as lowering of the brows beneath the superior orbital margins; (iii) vigorous frontalis contraction during periods of inability to raise eyelids; (iv) no oculomotor or ocular sympathetic dysfunction, or ocular myopathy) [4]. No patient displayed dystonia in other body regions, or sensory tricks that reverted eyelid spasms. Although several patients received psychoactive drugs, the time course of involuntary eyelid closure excluded a diagnosis of drug-induced blepharospasm. A detailed presentation of the clinical features of these patients may be found in the Supplementary data. The category “documented psychogenic dystonia” is applied when dystonic symptoms are persistently relieved by psychotherapy, suggestion or placebo [2,5], or when the patient is witnessed free of symptoms when supposedly unobserved [2]. In this series, patients 1e4 experienced sustained relief of blepharospasm following http://dx.doi.org/10.1016/j.parkreldis.2014.12.005 1353-8020/© 2014 Elsevier Ltd. All rights reserved.
periodic administration of subcutaneous saline solution, and patient 4 was witnessed free of eyelid closure while supposedly unobserved. For these reasons, all of them could be ascribed to this group. Supplementary clues that suggested a psychogenic origin in this group were: 1) blepharospasm of sudden onset in patient 1; 2) changes in pattern and side of predominant eye closure in patient 2; 3) a decrease of eyelid closure during visual effort and when performing arithmetic calculations aloud, in patient 2; and 4) an absolute lack of response to treatments efficacious in blepharospasm (botulinum neurotoxin), in patients 1 and 4 [2,5]. The category “clinically established psychogenic dystonia plus other features” refers to abnormal movements inconsistent over time (i.e., spontaneous amelioration or remission of abnormal movements), or incongruous with the expression of organic dystonia (i.e., variability in the performance of involuntary movements, amelioration of dystonia during effort, bizarre movements), associated with psychogenic neurological signs, multiple somatizations or obvious psychiatric disturbances [2]. In this series, incongruous signs with respect to those of benign essential blepharospasm (eyelid narrowing in the absence of orbicularis oculi spasm) were found in patients 5 and 6; bizarre abnormal movements (bilateral eye convergence spasm) in patient 6, while inconsistency over time and non-stereotyped eyelid spasms became evident in patient 7. A diagnosis of psychiatric disease (conversion disorder, schizophrenia and major depression, respectively) had previously been made in each case; therefore, every one of them could be included in this class. The third category, “clinically established psychogenic dystonia minus other features”, refers to patients with unequivocal clinical features incompatible with organic disease, with no features suggesting another underlying neurological or psychiatric problem [2]. Patient 8 demonstrated inconsistency over time (spontaneous resolution of eyelid closure), and incongruity with essential blepharospasm (isolated contraction of procerus and corrugator muscles) in the absence of overt psychogenic or neurological disease, therefore fitting into this group. The three categories defined above are considered as “clinically definite” psychogenic dystonia [2]. Although our study group is too small to generate definitive conclusions, it is suggested from our results that the first objective of this study has been met, in that Gupta and Lang's guidelines to categorize psychogenic torsion dystonia [2], proved adequate for the diagnosis of isolated psychogenic blepharospasm in each patient of this series. On the opposite, specific clinical manifestations could not be identified for psychogenic blepharospasm. However, one motor sign found in this series, incongruous with the expression of essential blepharospasm, should draw attention to alternative aetiologies. This sign was the contraction of corrugator and procerus
326
Letter to the Editor / Parkinsonism and Related Disorders 21 (2015) 325e326
Table 1 Categories of psychogenic torsion dystonia, as defined by Gupta and Lang [3]. - Documented psychogenic dystonia Remittance with psychotherapy, suggestion, physiotherapy, or placebo. Dystonia absent while supposedly unobserved - Clinically established psychogenic dystonia, plus other features Inconsistent over time/incongruent with clinical condition plus other manifestations: obvious psychiatric disease, multiple somatizations, other “false” neurological signs - Clinically established psychogenic dystonia, minus other features Unequivocal clinical features incompatible with organic disease, with no features suggesting another underlying neurological or psychiatric problem
muscles (which causes narrowing of eyelid fissure and frowning of the eyebrows), in the absence of spasm of the orbicularis oculi. It was found in psychogenic blepharospasm in our patients, and may also be triggered by ophthalmic disease. Prognosis was favorable in this series, attesting to inconsistency over time of psychogenic blepharospasm. Spontaneous remission took place in four cases (50%), while the remaining patients experienced prolonged symptomatic relief from administration of placebo. It is worth mentioning that every patient in this series displayed isolated eyelid closure; if additional psychogenic neurological signs had been present, a diagnosis of functional movement disorder would have been more straightforward. Besides, no sensory tricks were reported, which was a significant clue for excluding essential blepharospasm, as these are considered highly specific features of dystonia [1]. Further research is needed to confirm our conclusions, and to adequately characterize the clinical characteristics of psychogenic blepharospasm.
Appendix A. Supplementary data Supplementary data related to this article can be found at http:// dx.doi.org/10.1016/j.parkreldis.2014.12.005.
References [1] Defazio G, Hallett M, Jinnah HA, Berardelli A. Development and validation of a guideline for diagnosing blepharospasm. Neurology 2013;81:236e40. [2] Gupta A, Lang AE. Psychogenic movement disorders. Curr Opin Neurol 2009;22: 430e6. [3] Fahn S, Williams DT. Psychogenic dystonia. Adv Neurol 1988;50:431e55. [4] Lepore FE, Duvoisin RC. “Apraxia” of eyelid opening: an involuntary levator inhibition. Neurology 1985;35:423e7. [5] Fasano A, Valadas A, Bathia KB, Prashanth DM, Lang AE, Munhoz RP, et al. Psychogenic facial movement disorders: clinical features and associated conditions. Mov Disord 2012;27:1544e51.
Gazulla*, Sebastia n García-Rubio Jose Department of Neurology, Hospital Universitario Miguel Servet, Zaragoza, Spain Carlota Ruiz-Gazulla Faculty of Biotechnology, University of Zaragoza, Zaragoza, Spain Pedro Modrego Department of Neurology, Hospital Universitario Miguel Servet, Zaragoza, Spain *
Corresponding author. Luis Vives 6, esc dcha, 7 B, 50006 Zaragoza, Spain. Tel.: þ34 976563649. E-mail address: [email protected] (J. Gazulla). 11 September 2014
