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Una Marie Sheerin - One of the best experts on this subject based on the ideXlab platform.
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Glycine Receptor antibodies in perm and related syndromes characteristics clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajalgonzalez, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Isabel M Leite, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (<1:50) and unknown or alternative diagnoses were excluded from further study. Three of the remaining 45 patients had newly-identified thymomas and one had a lymphoma. Thirty-three patients were classified as progressive encephalomyelitis with rigidity and myoclonus, and two as stiff person syndrome; five had a limbic encephalitis or epileptic encephalopathy, two had brainstem features mainly, two had demyelinating optic neuropathies and one had an unclear diagnosis. Four patients (9%) died during the acute disease, but most showed marked improvement with immunotherapies. At most recent follow-up, (2-7 years, median 3 years, since first antibody detection), the median modified Rankin scale scores (excluding the four deaths) decreased from 5 at maximal severity to 1 (P < 0.0001), but relapses have occurred in five patients and a proportion are on reducing steroids or other maintenance immunotherapies as well as symptomatic treatments. The Glycine Receptor antibodies activated complement on Glycine Receptor-transfected human embryonic kidney cells at room temperature, and caused internalization and lysosomal degradation of the Glycine Receptors at 37°C. Immunoglobulin G antibodies bound to rodent spinal cord and brainstem co-localizing with monoclonal antibodies to Glycine Receptor-α1. Ten Glycine Receptor antibody positive samples were also identified in a retrospective cohort of 56 patients with stiff person syndrome and related syndromes. Glycine Receptor antibodies are strongly associated with spinal and brainstem disorders, and the majority of patients have progressive encephalomyelitis with rigidity and myoclonus. The antibodies demonstrate in vitro evidence of pathogenicity and the patients respond well to immunotherapies, contrasting with earlier studies of this syndrome, which indicated a poor prognosis. The presence of Glycine Receptor antibodies should help to identify a disease that responds to immunotherapies, but these treatments may need to be sustained, relapses can occur and maintenance immunosuppression may be required.
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Glycine Receptor antibodies in PERM and related syndromes: Characteristics, clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajal-gonzález, M. Isabel Leite, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (
Patrick Waters - One of the best experts on this subject based on the ideXlab platform.
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Glycine Receptor antibodies in perm and related syndromes characteristics clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajalgonzalez, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Isabel M Leite, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (<1:50) and unknown or alternative diagnoses were excluded from further study. Three of the remaining 45 patients had newly-identified thymomas and one had a lymphoma. Thirty-three patients were classified as progressive encephalomyelitis with rigidity and myoclonus, and two as stiff person syndrome; five had a limbic encephalitis or epileptic encephalopathy, two had brainstem features mainly, two had demyelinating optic neuropathies and one had an unclear diagnosis. Four patients (9%) died during the acute disease, but most showed marked improvement with immunotherapies. At most recent follow-up, (2-7 years, median 3 years, since first antibody detection), the median modified Rankin scale scores (excluding the four deaths) decreased from 5 at maximal severity to 1 (P < 0.0001), but relapses have occurred in five patients and a proportion are on reducing steroids or other maintenance immunotherapies as well as symptomatic treatments. The Glycine Receptor antibodies activated complement on Glycine Receptor-transfected human embryonic kidney cells at room temperature, and caused internalization and lysosomal degradation of the Glycine Receptors at 37°C. Immunoglobulin G antibodies bound to rodent spinal cord and brainstem co-localizing with monoclonal antibodies to Glycine Receptor-α1. Ten Glycine Receptor antibody positive samples were also identified in a retrospective cohort of 56 patients with stiff person syndrome and related syndromes. Glycine Receptor antibodies are strongly associated with spinal and brainstem disorders, and the majority of patients have progressive encephalomyelitis with rigidity and myoclonus. The antibodies demonstrate in vitro evidence of pathogenicity and the patients respond well to immunotherapies, contrasting with earlier studies of this syndrome, which indicated a poor prognosis. The presence of Glycine Receptor antibodies should help to identify a disease that responds to immunotherapies, but these treatments may need to be sustained, relapses can occur and maintenance immunosuppression may be required.
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Glycine Receptor antibody mediated progressive encephalomyelitis with rigidity and myoclonus perm a rare but treatable neurological syndrome
Practical Neurology, 2014Co-Authors: W Stern, Mark Woodhall, Patrick Waters, Angela Vincent, Robert Howard, R M Chalmers, M WickremaratchiAbstract:A 40-year-old man presented with respiratory compromise and was intubated. After tracheostomy, he was found to have ophthalmoplegia, severe limb rigidity, stimulus-sensitive myoclonus and autonomic dysfunction. For 1eweek before admission, there had been a prodromal illness with low mood, hallucinations and limb myoclonus. Serum Glycine Receptor antibodies were strongly positive: we diagnosed progressive encephalomyelitis with rigidity and myoclonus. Despite a relapse, he has done well following immunotherapies. The clinical syndrome of encephalomyelitis with rigidity, described in 1976, often has a severe progressive course. A minority of patients have glutamic acid decarboxylase antibodies. The association with Glycine Receptor antibody was first reported in 2008, and we briefly review subsequent case reports to illustrate the range of clinical features. The antibody is likely to be disease mediating, although this remains unproven. The spectrum of diagnosable and treatable antibody mediated neurological syndromes is expanding. It is vital to recognise these conditions early to reduce morbidity and mortality.
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Glycine Receptor antibodies in PERM and related syndromes: Characteristics, clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajal-gonzález, M. Isabel Leite, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (
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progressive encephalomyelitis with rigidity and myoclonus the first pediatric case with Glycine Receptor antibodies
JAMA Neurology, 2013Co-Authors: Joana Damasio, Ester Coutinho, Mark Woodhall, Patrick Waters, Maria Isabel Leite, M A Santos, I Carrilho, Angela VincentAbstract:Importance Progressive encephalomyelitis with rigidity and myoclonus is characterized by rigidity, painful muscle spasms, hyperekplexia, and brainstem signs. Recently, Glycine Receptor alpha 1 antibodies have been described in adult patients with progressive encephalomyelitis with rigidity and myoclonus. We describe a pediatric case. Observations A 14-month-old child developed startle-induced episodes of generalized rigidity and myoclonus, axial hyperextension, and trismus, without impairment of consciousness. Episodes occurred during wakefulness and sleep, lasted seconds, and were accompanied by moaning, tachypnea, and oxygen desaturation. Imaging, cerebrospinal fluid, endocrine, metabolic, and genetic screening findings were normal or negative. She was treated with intravenous steroids and immunoglobulins with resolution of symptoms, but she relapsed weeks later. At this time, episodes were more severe. Glycine Receptor alpha 1 antibodies were found in serum (titer of 1:200, later 1:320) and cerebrospinal fluid (titer of 1:2). Treatment was restarted with intravenous steroids and immunoglobulins, with major improvement, and she began treatment with oral steroids. She had 4 milder relapses, with improvement after treatment adjustments. Conclusions and Relevance To our knowledge, this is the first pediatric case of progressive encephalomyelitis with rigidity and myoclonus associated with Glycine Receptor alpha 1 antibodies, a potentially severe but treatable antibody-mediated neurological disorder.
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progressive encephalomyelitis rigidity and myoclonus a novel Glycine Receptor antibody
Neurology, 2008Co-Authors: Michael Hutchinson, Patrick Waters, Cord-michael Becker, John C Mchugh, Grainne S Gorman, Sean Oriordan, S Connolly, H Hager, Angela VincentAbstract:Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disorder of subacute onset presenting as limb and truncal rigidity, muscle spasms, brainstem signs, and hyperekplexia.1 Life-threatening, it is part of the spectrum of stiff-person syndrome (SPS) with anti-glutamic acid decarboxylase (GAD) antibodies in up to 80% of patients.1 It may also be paraneoplastic with anti-amphiphysin antibodies.2 Mutations in the α1 subunit of the Glycine Receptor gene GLRA1 have been identified in hereditary hyperekplexia.3 Glycine Receptors are concentrated in the caudal pontine brainstem and spinal cord; in PERM, hyperekplexia (brainstem myoclonus or excessive startle)4 implies disruption of the normal inhibitory Glycinergic mechanism in the caudal pons where the nucleus reticularis pontis caudalis mediates the startle reflex. We report a patient with typical severe PERM associated with a novel anti-Glycine Receptor antibody. ### Clinical summary. A 54-year-old man presented in January 2001 with 3 weeks of left flank tingling and 2 weeks of worsening brief frequent violent jerks, spontaneous and triggered by sensory and auditory stimuli; his upper limbs would abduct and flex and his trunk and legs extend. Neurologic examination was otherwise normal; he was intubated and ventilated. The CSF contained 60 lymphocytes × 109/L, the CSF IgG index was 0.72 (n < 0.7), oligoclonal bands negative. Cranial and spinal MRI scans with gadolinium were normal. After …
Mark Woodhall - One of the best experts on this subject based on the ideXlab platform.
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Glycine Receptor antibodies in perm and related syndromes characteristics clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajalgonzalez, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Isabel M Leite, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (<1:50) and unknown or alternative diagnoses were excluded from further study. Three of the remaining 45 patients had newly-identified thymomas and one had a lymphoma. Thirty-three patients were classified as progressive encephalomyelitis with rigidity and myoclonus, and two as stiff person syndrome; five had a limbic encephalitis or epileptic encephalopathy, two had brainstem features mainly, two had demyelinating optic neuropathies and one had an unclear diagnosis. Four patients (9%) died during the acute disease, but most showed marked improvement with immunotherapies. At most recent follow-up, (2-7 years, median 3 years, since first antibody detection), the median modified Rankin scale scores (excluding the four deaths) decreased from 5 at maximal severity to 1 (P < 0.0001), but relapses have occurred in five patients and a proportion are on reducing steroids or other maintenance immunotherapies as well as symptomatic treatments. The Glycine Receptor antibodies activated complement on Glycine Receptor-transfected human embryonic kidney cells at room temperature, and caused internalization and lysosomal degradation of the Glycine Receptors at 37°C. Immunoglobulin G antibodies bound to rodent spinal cord and brainstem co-localizing with monoclonal antibodies to Glycine Receptor-α1. Ten Glycine Receptor antibody positive samples were also identified in a retrospective cohort of 56 patients with stiff person syndrome and related syndromes. Glycine Receptor antibodies are strongly associated with spinal and brainstem disorders, and the majority of patients have progressive encephalomyelitis with rigidity and myoclonus. The antibodies demonstrate in vitro evidence of pathogenicity and the patients respond well to immunotherapies, contrasting with earlier studies of this syndrome, which indicated a poor prognosis. The presence of Glycine Receptor antibodies should help to identify a disease that responds to immunotherapies, but these treatments may need to be sustained, relapses can occur and maintenance immunosuppression may be required.
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Glycine Receptor antibody mediated progressive encephalomyelitis with rigidity and myoclonus perm a rare but treatable neurological syndrome
Practical Neurology, 2014Co-Authors: W Stern, Mark Woodhall, Patrick Waters, Angela Vincent, Robert Howard, R M Chalmers, M WickremaratchiAbstract:A 40-year-old man presented with respiratory compromise and was intubated. After tracheostomy, he was found to have ophthalmoplegia, severe limb rigidity, stimulus-sensitive myoclonus and autonomic dysfunction. For 1eweek before admission, there had been a prodromal illness with low mood, hallucinations and limb myoclonus. Serum Glycine Receptor antibodies were strongly positive: we diagnosed progressive encephalomyelitis with rigidity and myoclonus. Despite a relapse, he has done well following immunotherapies. The clinical syndrome of encephalomyelitis with rigidity, described in 1976, often has a severe progressive course. A minority of patients have glutamic acid decarboxylase antibodies. The association with Glycine Receptor antibody was first reported in 2008, and we briefly review subsequent case reports to illustrate the range of clinical features. The antibody is likely to be disease mediating, although this remains unproven. The spectrum of diagnosable and treatable antibody mediated neurological syndromes is expanding. It is vital to recognise these conditions early to reduce morbidity and mortality.
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Glycine Receptor antibodies in PERM and related syndromes: Characteristics, clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajal-gonzález, M. Isabel Leite, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (
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progressive encephalomyelitis with rigidity and myoclonus the first pediatric case with Glycine Receptor antibodies
JAMA Neurology, 2013Co-Authors: Joana Damasio, Ester Coutinho, Mark Woodhall, Patrick Waters, Maria Isabel Leite, M A Santos, I Carrilho, Angela VincentAbstract:Importance Progressive encephalomyelitis with rigidity and myoclonus is characterized by rigidity, painful muscle spasms, hyperekplexia, and brainstem signs. Recently, Glycine Receptor alpha 1 antibodies have been described in adult patients with progressive encephalomyelitis with rigidity and myoclonus. We describe a pediatric case. Observations A 14-month-old child developed startle-induced episodes of generalized rigidity and myoclonus, axial hyperextension, and trismus, without impairment of consciousness. Episodes occurred during wakefulness and sleep, lasted seconds, and were accompanied by moaning, tachypnea, and oxygen desaturation. Imaging, cerebrospinal fluid, endocrine, metabolic, and genetic screening findings were normal or negative. She was treated with intravenous steroids and immunoglobulins with resolution of symptoms, but she relapsed weeks later. At this time, episodes were more severe. Glycine Receptor alpha 1 antibodies were found in serum (titer of 1:200, later 1:320) and cerebrospinal fluid (titer of 1:2). Treatment was restarted with intravenous steroids and immunoglobulins, with major improvement, and she began treatment with oral steroids. She had 4 milder relapses, with improvement after treatment adjustments. Conclusions and Relevance To our knowledge, this is the first pediatric case of progressive encephalomyelitis with rigidity and myoclonus associated with Glycine Receptor alpha 1 antibodies, a potentially severe but treatable antibody-mediated neurological disorder.
Ester Coutinho - One of the best experts on this subject based on the ideXlab platform.
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Glycine Receptor antibodies in perm and related syndromes characteristics clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajalgonzalez, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Isabel M Leite, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (<1:50) and unknown or alternative diagnoses were excluded from further study. Three of the remaining 45 patients had newly-identified thymomas and one had a lymphoma. Thirty-three patients were classified as progressive encephalomyelitis with rigidity and myoclonus, and two as stiff person syndrome; five had a limbic encephalitis or epileptic encephalopathy, two had brainstem features mainly, two had demyelinating optic neuropathies and one had an unclear diagnosis. Four patients (9%) died during the acute disease, but most showed marked improvement with immunotherapies. At most recent follow-up, (2-7 years, median 3 years, since first antibody detection), the median modified Rankin scale scores (excluding the four deaths) decreased from 5 at maximal severity to 1 (P < 0.0001), but relapses have occurred in five patients and a proportion are on reducing steroids or other maintenance immunotherapies as well as symptomatic treatments. The Glycine Receptor antibodies activated complement on Glycine Receptor-transfected human embryonic kidney cells at room temperature, and caused internalization and lysosomal degradation of the Glycine Receptors at 37°C. Immunoglobulin G antibodies bound to rodent spinal cord and brainstem co-localizing with monoclonal antibodies to Glycine Receptor-α1. Ten Glycine Receptor antibody positive samples were also identified in a retrospective cohort of 56 patients with stiff person syndrome and related syndromes. Glycine Receptor antibodies are strongly associated with spinal and brainstem disorders, and the majority of patients have progressive encephalomyelitis with rigidity and myoclonus. The antibodies demonstrate in vitro evidence of pathogenicity and the patients respond well to immunotherapies, contrasting with earlier studies of this syndrome, which indicated a poor prognosis. The presence of Glycine Receptor antibodies should help to identify a disease that responds to immunotherapies, but these treatments may need to be sustained, relapses can occur and maintenance immunosuppression may be required.
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Glycine Receptor antibodies in PERM and related syndromes: Characteristics, clinical features and outcomes
Brain, 2014Co-Authors: Alexander Carvajal-gonzález, M. Isabel Leite, Aisling Carr, Ester Coutinho, Bethan Lang, Mark Woodhall, Patrick Waters, Philippa Pettingill, Bettina Balint, Una Marie SheerinAbstract:The clinical associations of Glycine Receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum Glycine Receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of Glycine Receptor antibodies in each of the six pairs available for detailed study. Four patients also had high glutamic acid decarboxylase antibodies (>1000 U/ml), and one had high voltage-gated potassium channel-complex antibody (2442 pM). Seven patients with very low titres (
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progressive encephalomyelitis with rigidity and myoclonus the first pediatric case with Glycine Receptor antibodies
JAMA Neurology, 2013Co-Authors: Joana Damasio, Ester Coutinho, Mark Woodhall, Patrick Waters, Maria Isabel Leite, M A Santos, I Carrilho, Angela VincentAbstract:Importance Progressive encephalomyelitis with rigidity and myoclonus is characterized by rigidity, painful muscle spasms, hyperekplexia, and brainstem signs. Recently, Glycine Receptor alpha 1 antibodies have been described in adult patients with progressive encephalomyelitis with rigidity and myoclonus. We describe a pediatric case. Observations A 14-month-old child developed startle-induced episodes of generalized rigidity and myoclonus, axial hyperextension, and trismus, without impairment of consciousness. Episodes occurred during wakefulness and sleep, lasted seconds, and were accompanied by moaning, tachypnea, and oxygen desaturation. Imaging, cerebrospinal fluid, endocrine, metabolic, and genetic screening findings were normal or negative. She was treated with intravenous steroids and immunoglobulins with resolution of symptoms, but she relapsed weeks later. At this time, episodes were more severe. Glycine Receptor alpha 1 antibodies were found in serum (titer of 1:200, later 1:320) and cerebrospinal fluid (titer of 1:2). Treatment was restarted with intravenous steroids and immunoglobulins, with major improvement, and she began treatment with oral steroids. She had 4 milder relapses, with improvement after treatment adjustments. Conclusions and Relevance To our knowledge, this is the first pediatric case of progressive encephalomyelitis with rigidity and myoclonus associated with Glycine Receptor alpha 1 antibodies, a potentially severe but treatable antibody-mediated neurological disorder.
Marc H. De Baets - One of the best experts on this subject based on the ideXlab platform.
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Glycine Receptor antibodies in PERM: a new channelopathy
Brain, 2014Co-Authors: Pilar Martinez-martinez, Peter C. M. Molenaar, Mario Losen, Marc H. De BaetsAbstract:This scientific commentary refers to ‘Glycine Receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes', by Carvajal-Gonzalez et al. (doi:10.1093/brain/awu142).