- Email: [email protected]
Peri-oral myoclonia with absences with multiple facial and upper body myoclonia: Overlap epilepsy syndrome
Case Reports / Journal of Clinical Neuroscience 54 (2018) 135–137 [58] Baehring JM, Alemohammed S, Croul SE. Malignant fibrous histiocytoma presenting as an intraventricular mass 5 years after incidental detection of a mass lesion. J. Neuro-Oncol. 2001;52:157–60. [59] Berchtenbreiter CHG, Stehling MK, Scheidler J, Bise K, Waidhauser E, Reiser M. CT and MRI findings with pathological correlation of an intracerebral malignant fibrous histiocytoma (MFH): a case report. Eur. Radiol. 1996;6:910–2. [60] Galatioto S, Gullota F. Peculiar meningeal tumour in a 4-month old baby: malignant fibrous histiocytoma? Pathologica 1995;87(1):74–7. [61] Hamlat A, Adn M, Caulet-Maugendre S, Guegan Y. Cerebellar malignant fibrous histiocytoma: case report and literature review. Neurosurgery 2004;54:745–52. [62] Ho YS, Wei CH, Tsai MD, et al. Intracerebral malignant fibrous histiocytoma: case report and review of the literature. Neurosurgery 1992;31:567–71. [63] Lam RM, Colah SA. Atypical fibrous histiocytoma with myxoid stroma: a rare lesion arising from the dura mater of the brain. Cancer 1979;43:237–45.
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[64] Ogino A, Ochi M, Hayashi K, et al. MRI of intracranial meningeal malignant fibrous histiocytoma. Neuroradiology 1996;38:785–7. [65] Ozhan S, Tali ET, Isik S, Saygili MR, Baykaner K. Haematoma-like primary intracranial malignant fibrous histiocytoma in a 5-year old girl. Neuroradiology 1999;41:523–5. [66] Romero FJ, Ortega A, Ibarra B, Piqueras J, Rovira M. Post-radiation cranial malignant fibrous histiocytoma studied by CT. Comput. Med. Imaging Graph. 1989;13:191–4. [67] Schrader B, Holland BR, Friedrichsen C. Rare case of a primary malignant fibrous histiocytoma. Neuroradiology 1989;31:177–9. [68] Yoo R, Choi SH, Park S, et al. Primary intracerebral malignant fibrous histiocytoma: CT, MRI, and PET-CT findings. J. Neuroimag. 2013;23:141–4. [69] Yoshikai T, Shimokawa S, Uchino A, et al. Thallium-201 SPECT of adjacent intracranial tumours: a contrast in thallium kinetics. Neuroradiology 1999;41:646–9.
https://doi.org/10.1016/j.jocn.2018.04.060
Peri-oral myoclonia with absences with multiple facial and upper body myoclonia: Overlap epilepsy syndrome Pratima Gulati a, Puneet Jain a, Hebah Qashqari a, Laura Bradbury a, Pamela Cooper b, Hiroshi Otsubo a, Robyn Whitney a,⇑ a b
Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada Community Pediatric Neurologist, Stouffville, Ontario, Canada
a r t i c l e
i n f o
Article history: Received 13 March 2018 Accepted 4 June 2018
Keywords: POMA Jeavons syndrome Absences EEG Eye closure sensitivity
a b s t r a c t A 17-years old girl presented with an 8-year history of absences with peri-oral twitching, eyelid twitching and head nodding, with poor response to anti-epileptic drugs. Video EEG revealed ictal and inter-ictal generalized spike wave discharges, and absences with peri-oral (predominant), eyelid, neck and shoulder myoclonia. There was also prominent eye closure sensitivity. Conundrums regarding epilepsy syndrome classification and pathophysiology are discussed. Crown Copyright Ó 2018 Published by Elsevier Ltd. All rights reserved.
1. Main text 1.1. Description A 17-year old developmentally-normal girl presented with 8 years of paroxysmal daily, multiple, brief episodes of peri-oral twitching lasting up to 6 s with occasional head nodding during the day. There was also a history of daydreaming and inattention at school during this period. These were managed conservatively initially by the treating general physician. She was first referred to a pediatric neurologist at 12 years of age after her first generalized tonic-clonic seizure lasting up to 5 min. Electroencephalogram (EEG) at the time showed a single paroxysm of bifrontal polyspike-wave activity. She was commenced on Levetiracetam. Over the next three years, she had five more generalized tonic-clonic seizures lasting for less than 5 min. Lack of sleep, emotional or physical stress were reported as sei-
⇑ Corresponding author. Division of Neurology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. E-mail addresses: [email protected] (P. Gulati), [email protected] (P. Jain), [email protected] (H. Qashqari), [email protected] (L. Bradbury), [email protected] (P. Cooper), [email protected] (H. Otsubo), [email protected] (R. Whitney).
zures triggers. A repeat EEG showed infrequent left frontal spike and wave discharges. Carbamazepine was added due to the suspicion of focal seizures. Subsequently, she developed more frequent episodes of brief behavioral arrest followed by brief right sided facial twitching. Her history was negative for early morning jerks/clumsiness and recurrent falls. There was no history of other seizure types and the family history was non-contributory. She had normal neurological examination and magnetic resonance imaging of the brain. Prolonged video-EEG was requested to characterize the ongoing events and guide anticonvulsant drug therapy. Her EEG is shown in Fig. 1. The background activity was within normal limits for age and hyperventilation and photic stimulation were unremarkable. The inter-ictal EEG was composed of 3.5 Hz generalized spikes and wave, which were of similar morphology to the ictal EEG pattern but briefer. During the video-EEG, multiple episodes of absence seizures with myoclonic manifestations which included peri-oral (Video 1), eyelid, neck and shoulder myoclonia were captured. Eye-closure sensitivity was prominent. A diagnosis of peri-oral myoclonia with absence epilepsy was made (POMA) in view of predominant myoclonia in the peri-oral region. Ethosuximide was added and Carbamazepine was weaned. She continued to have ongoing facial twitching at the last follow up.
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Case Reports / Journal of Clinical Neuroscience 54 (2018) 135–137
Video 1. Extract from the video-EEG of the patient. The video shows three separate brief seizures characterized by peri-oral myoclonia. There was associated 3–4 Hz generalized spike wave discharges on EEG. There were also events with eyelid, neck and shoulder myoclonia (not shown here).
2. Discussion Perioral myoclonia with absences (POMA) was first described in 1994 by Panayiotopoulos (4). It is characterized by onset between 2 and 13 years of age, absences with myoclonia of the peri-oral muscles, co-existing generalized tonic-clonic seizures, propensity to absence status and poor response to anti-epileptic drugs. EEG shows ictal and inter-ictal generalized irregular 3–4 Hz spike/polyspike wave discharges, inconsistent correlation of myoclonia with spike wave activity, frequent focal abnormalities and the absence of photosensitivity [1,2]. Asymmetric facial twitching and focal EEG abnormalities led to a misdiagnosis of a focal epilepsy in our case. Further seizure aggravation with sodium channel blockers like carbamazepine is also known [3].
Eye closure sensitivity refers to the transient appearance of epileptiform discharges on EEG (usually generalized) within 2–4 s after eye closure. It is an important diagnostic feature for eyelid myoclonia with absences (EMA) also known as Jeavons Syndrome. However, it is not specific and has been reported in other genetic generalized epilepsies along with focal occipital epilepsies [4]. Some authors believed that eye closure sensitivity may suggest poor prognosis for absence epilepsies but the results have been contradictory [4,5]. It has not been described in association with POMA before. However, presence of eyelid myoclonia along with eye closure sensitivity in our case may imply a unique overlap of POMA and EMA. Capovilla et al. [6] described children with typical absence seizures associated with myoclonic jerks. Myoclonia affecting eyebrows (4), peri-oral region (4), nostrils (1), chin (1) and neck (2) were reported. The majority of these cases had good seizure outcomes and an electro-clinical picture consistent with childhood absence epilepsy. Our case demonstrated myoclonia affecting peri-oral muscles, eyelids, neck and shoulder muscles. The exact mechanism underlying these myoclonic manifestations is unclear. The three critical areas for generation of absence seizures are believed to be the cerebral cortex (possibly anterior and mesial frontal cortex), thalamocortical relay cells and reticular thalamic nuclei [7]. The cortical focus theory [8] proposed that the peri-oral part of the somato-sensory cortex produces the initial spike which rapidly spreads over the cortex and then involve the thalamocortical network. Initially, the cortex drives the thalamus but subsequently thalamus and cortex start to drive each other, thus maintaining the discharges. Rozendaal et al. [9] demonstrated focal inter-ictal epileptiform discharges (frontal, central or parietal) in children with absence epilepsy on magnetoencephalography. It is possible that focal cortical dysfunction can produce various focal myoclonic manifestations depending on the location.
Fig. 1. EEG of the patient. The EEG shows high amplitude generalized 3.5 Hz spike wave discharges lasting for 3.5 s. This was associated with brief absence with peri-oral myoclonia. Note that this bursts follows the eye-closure within 400 msec. Hyperventilation and photic stimulation were unremarkable. The inter-ictal generalized spikes waves were of similar morphology but briefer. They often showed eye closure sensitivity. The background was age appropriate. (Bipolar longitudinal montage, LFF 1 Hz; HFF 70 Hz; sensitivity 15 mV/mm; timebase 30 mm/sec; sampling rate 256 Hz).
Case Reports / Journal of Clinical Neuroscience 54 (2018) 137–139
The exact epilepsy syndrome classification may be difficult in our case. Given the predominant peri-oral myoclonia, frequent generalized tonic-clonic seizures, poor response to anti-epileptic drugs, eye closure sensitivity and presence of other myoclonia, a diagnosis of POMA overlapping with EMA was considered. With increasing use of video-EEG monitoring across the world, more such cases are expected to be reported with further evolution of the epilepsy syndrome classification. Further, the better understanding of the neurophysiology of absence seizures may also influence their classification. 3. Conflicts of Interests/Sources of support None. 4. Ethics Informed consent was taken from the parents for publication of this case report along with videos. Acknowledgements The authors are grateful to the EEG technicians at The Hospital for Sick Children who helped in the acquisition of the EEG.
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References [1] Rubboli G, Gardella E, Capovilla G. Idiopathic generalized epilepsy (IGE) syndromes in development: IGE with absences of early childhood, IGE with phantom absences, and perioral myoclonia with absences. Epilepsia 2009;50 (suppl 5):24–8. [2] d’Orsi G, Demaio V, Trivisano M, Pascarella MG, Specchio LM. Ictal videopolygraphic features of perioral myoclonia with absences. Epilepsy Behav 2011;21:314–7. [3] Vrielynck P, Rostomashvili N, Degroote E, Ghariani S, van Rijckevorsel K. Perioral myoclonia with absences and myoclonic status aggravated by oxcarbazepine. Epileptic Disord 2011;13:308–12. [4] Sevgi EB, Saygi S, Ciger A. Eye closure sensitivity and epileptic syndromes: a retrospective study of 26 adult cases. Seizure 2007;16:17–21. [5] Tekin Güveli B, Baykan B, Dörtcan N, Bebek N, Gürses C, Gökyig˘it A. Eye closure sensitivity in juvenile myoclonic epilepsy and its effect on prognosis. Seizure 2013;22:867–71. [6] Cappovilla G, Rubboli G, Beccaria F, et al. A clinical spectrum of the myoclonic manifestations associated with typical absences in childhood absence epilepsy. A video-polygraphic study. Epileptic Disord 2001;3:57–61. [7] Craiu D, Magureanu S, van Emde Boas W. Are absences truly generalized seizures or partial seizures originating from or predominantlyinvolving the premotor areas? Some clinical and theoretical observations and their implicationsfor seizure classification. Epilepsy Res 2006;70(suppl 1):S141–55. [8] Meeren H, van Luijtelaar G, Lopes da Silva F, Coenen A. Evolving concepts on the pathophysiology of absence seizures: the cortical focus theory. Arch Neurol 2005;62:371–6. [9] Rozendaal YJ, van Luijtelaar G, Ossenblok PP. Spatiotemporal mapping of interictal epileptiform discharges in human absence epilepsy: a MEG study. Epilepsy Res 2016;119:67–76.
https://doi.org/10.1016/j.jocn.2018.06.008
Effects of cognitive and motor rehabilitation in non-convulsive status epilepticus: A case report Francesco Corallo a,⇑, Viviana Lo Buono a, Marcella Di Cara a, Simona De Salvo a, Giuliana Vermiglio a, Patrizia Pollicino a, Placido Bramanti a, Silvia Marino a,b, Carmela Rifici a a b
IRCCS Centro Neurolesi ‘‘Bonino-Pulejo”, Messina, Italy Department of Biomedical and Dental Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
a r t i c l e
i n f o
Article history: Received 7 February 2018 Accepted 6 June 2018
Keywords: Cognitive impairment Cognitive rehabilitation Non-convulsive status epilepticus
a b s t r a c t Epilepsy is a neurological disorder characterized by recurrent and unpredictable interruptions of normal brain function, that implies neurobiologic, psychological, and social consequences. Non-convulsive status epilepticus (NCSE) is an electro-clinical state associated to altered level of consciousness. In NCSE could occur the behavioural and emotional disorder, cognitive impairment and psychiatric disorder with a negative impact on quality of life and adaptive social behaviours. Cognitive rehabilitation for epileptic patients may include internal compensation strategies, external memory aids, psychoeducation, verbal and visual memory training, attention exercise and executive functions. We described the cognitive rehabilitation in a patient with NCSE characterized by an unusual cognitive impairment. A 55-year-old female patient, with epileptic seizures presented a severe impairment in cognitive function and focal neurological deficit. The cognitive treatment was designed as a combination of direct training of the impaired functions and metacognitive training to facilitate the development of compensatory strategies and it was divided into three methods. Neuropsychological evaluation showed a progressive deficit of high cognitive functions. The patient after cognitive rehabilitation, demonstrated a significant recovery of motor abilities, despite the neurocognitive profile was abnormal. Several advances have been made in this field but few studies on neuropsychological rehabilitation in adult patients with NCSE have been conducted and no standardized protocol or clinical guidelines are still available. Ó 2018 Elsevier Ltd. All rights reserved.
⇑ Corresponding author at: IRCCS Centro Neurolesi ‘‘Bonino-Pulejo”, S.S. 113 Via Palermo, C.da Casazza, 98124 Messina, Italy. E-mail address: [email protected] (F. Corallo).
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[64] Ogino A, Ochi M, Hayashi K, et al. MRI of intracranial meningeal malignant fibrous histiocytoma. Neuroradiology 1996;38:785–7. [65] Ozhan S, Tali ET, Isik S, Saygili MR, Baykaner K. Haematoma-like primary intracranial malignant fibrous histiocytoma in a 5-year old girl. Neuroradiology 1999;41:523–5. [66] Romero FJ, Ortega A, Ibarra B, Piqueras J, Rovira M. Post-radiation cranial malignant fibrous histiocytoma studied by CT. Comput. Med. Imaging Graph. 1989;13:191–4. [67] Schrader B, Holland BR, Friedrichsen C. Rare case of a primary malignant fibrous histiocytoma. Neuroradiology 1989;31:177–9. [68] Yoo R, Choi SH, Park S, et al. Primary intracerebral malignant fibrous histiocytoma: CT, MRI, and PET-CT findings. J. Neuroimag. 2013;23:141–4. [69] Yoshikai T, Shimokawa S, Uchino A, et al. Thallium-201 SPECT of adjacent intracranial tumours: a contrast in thallium kinetics. Neuroradiology 1999;41:646–9.
https://doi.org/10.1016/j.jocn.2018.04.060
Peri-oral myoclonia with absences with multiple facial and upper body myoclonia: Overlap epilepsy syndrome Pratima Gulati a, Puneet Jain a, Hebah Qashqari a, Laura Bradbury a, Pamela Cooper b, Hiroshi Otsubo a, Robyn Whitney a,⇑ a b
Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada Community Pediatric Neurologist, Stouffville, Ontario, Canada
a r t i c l e
i n f o
Article history: Received 13 March 2018 Accepted 4 June 2018
Keywords: POMA Jeavons syndrome Absences EEG Eye closure sensitivity
a b s t r a c t A 17-years old girl presented with an 8-year history of absences with peri-oral twitching, eyelid twitching and head nodding, with poor response to anti-epileptic drugs. Video EEG revealed ictal and inter-ictal generalized spike wave discharges, and absences with peri-oral (predominant), eyelid, neck and shoulder myoclonia. There was also prominent eye closure sensitivity. Conundrums regarding epilepsy syndrome classification and pathophysiology are discussed. Crown Copyright Ó 2018 Published by Elsevier Ltd. All rights reserved.
1. Main text 1.1. Description A 17-year old developmentally-normal girl presented with 8 years of paroxysmal daily, multiple, brief episodes of peri-oral twitching lasting up to 6 s with occasional head nodding during the day. There was also a history of daydreaming and inattention at school during this period. These were managed conservatively initially by the treating general physician. She was first referred to a pediatric neurologist at 12 years of age after her first generalized tonic-clonic seizure lasting up to 5 min. Electroencephalogram (EEG) at the time showed a single paroxysm of bifrontal polyspike-wave activity. She was commenced on Levetiracetam. Over the next three years, she had five more generalized tonic-clonic seizures lasting for less than 5 min. Lack of sleep, emotional or physical stress were reported as sei-
⇑ Corresponding author. Division of Neurology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. E-mail addresses: [email protected] (P. Gulati), [email protected] (P. Jain), [email protected] (H. Qashqari), [email protected] (L. Bradbury), [email protected] (P. Cooper), [email protected] (H. Otsubo), [email protected] (R. Whitney).
zures triggers. A repeat EEG showed infrequent left frontal spike and wave discharges. Carbamazepine was added due to the suspicion of focal seizures. Subsequently, she developed more frequent episodes of brief behavioral arrest followed by brief right sided facial twitching. Her history was negative for early morning jerks/clumsiness and recurrent falls. There was no history of other seizure types and the family history was non-contributory. She had normal neurological examination and magnetic resonance imaging of the brain. Prolonged video-EEG was requested to characterize the ongoing events and guide anticonvulsant drug therapy. Her EEG is shown in Fig. 1. The background activity was within normal limits for age and hyperventilation and photic stimulation were unremarkable. The inter-ictal EEG was composed of 3.5 Hz generalized spikes and wave, which were of similar morphology to the ictal EEG pattern but briefer. During the video-EEG, multiple episodes of absence seizures with myoclonic manifestations which included peri-oral (Video 1), eyelid, neck and shoulder myoclonia were captured. Eye-closure sensitivity was prominent. A diagnosis of peri-oral myoclonia with absence epilepsy was made (POMA) in view of predominant myoclonia in the peri-oral region. Ethosuximide was added and Carbamazepine was weaned. She continued to have ongoing facial twitching at the last follow up.
136
Case Reports / Journal of Clinical Neuroscience 54 (2018) 135–137
Video 1. Extract from the video-EEG of the patient. The video shows three separate brief seizures characterized by peri-oral myoclonia. There was associated 3–4 Hz generalized spike wave discharges on EEG. There were also events with eyelid, neck and shoulder myoclonia (not shown here).
2. Discussion Perioral myoclonia with absences (POMA) was first described in 1994 by Panayiotopoulos (4). It is characterized by onset between 2 and 13 years of age, absences with myoclonia of the peri-oral muscles, co-existing generalized tonic-clonic seizures, propensity to absence status and poor response to anti-epileptic drugs. EEG shows ictal and inter-ictal generalized irregular 3–4 Hz spike/polyspike wave discharges, inconsistent correlation of myoclonia with spike wave activity, frequent focal abnormalities and the absence of photosensitivity [1,2]. Asymmetric facial twitching and focal EEG abnormalities led to a misdiagnosis of a focal epilepsy in our case. Further seizure aggravation with sodium channel blockers like carbamazepine is also known [3].
Eye closure sensitivity refers to the transient appearance of epileptiform discharges on EEG (usually generalized) within 2–4 s after eye closure. It is an important diagnostic feature for eyelid myoclonia with absences (EMA) also known as Jeavons Syndrome. However, it is not specific and has been reported in other genetic generalized epilepsies along with focal occipital epilepsies [4]. Some authors believed that eye closure sensitivity may suggest poor prognosis for absence epilepsies but the results have been contradictory [4,5]. It has not been described in association with POMA before. However, presence of eyelid myoclonia along with eye closure sensitivity in our case may imply a unique overlap of POMA and EMA. Capovilla et al. [6] described children with typical absence seizures associated with myoclonic jerks. Myoclonia affecting eyebrows (4), peri-oral region (4), nostrils (1), chin (1) and neck (2) were reported. The majority of these cases had good seizure outcomes and an electro-clinical picture consistent with childhood absence epilepsy. Our case demonstrated myoclonia affecting peri-oral muscles, eyelids, neck and shoulder muscles. The exact mechanism underlying these myoclonic manifestations is unclear. The three critical areas for generation of absence seizures are believed to be the cerebral cortex (possibly anterior and mesial frontal cortex), thalamocortical relay cells and reticular thalamic nuclei [7]. The cortical focus theory [8] proposed that the peri-oral part of the somato-sensory cortex produces the initial spike which rapidly spreads over the cortex and then involve the thalamocortical network. Initially, the cortex drives the thalamus but subsequently thalamus and cortex start to drive each other, thus maintaining the discharges. Rozendaal et al. [9] demonstrated focal inter-ictal epileptiform discharges (frontal, central or parietal) in children with absence epilepsy on magnetoencephalography. It is possible that focal cortical dysfunction can produce various focal myoclonic manifestations depending on the location.
Fig. 1. EEG of the patient. The EEG shows high amplitude generalized 3.5 Hz spike wave discharges lasting for 3.5 s. This was associated with brief absence with peri-oral myoclonia. Note that this bursts follows the eye-closure within 400 msec. Hyperventilation and photic stimulation were unremarkable. The inter-ictal generalized spikes waves were of similar morphology but briefer. They often showed eye closure sensitivity. The background was age appropriate. (Bipolar longitudinal montage, LFF 1 Hz; HFF 70 Hz; sensitivity 15 mV/mm; timebase 30 mm/sec; sampling rate 256 Hz).
Case Reports / Journal of Clinical Neuroscience 54 (2018) 137–139
The exact epilepsy syndrome classification may be difficult in our case. Given the predominant peri-oral myoclonia, frequent generalized tonic-clonic seizures, poor response to anti-epileptic drugs, eye closure sensitivity and presence of other myoclonia, a diagnosis of POMA overlapping with EMA was considered. With increasing use of video-EEG monitoring across the world, more such cases are expected to be reported with further evolution of the epilepsy syndrome classification. Further, the better understanding of the neurophysiology of absence seizures may also influence their classification. 3. Conflicts of Interests/Sources of support None. 4. Ethics Informed consent was taken from the parents for publication of this case report along with videos. Acknowledgements The authors are grateful to the EEG technicians at The Hospital for Sick Children who helped in the acquisition of the EEG.
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References [1] Rubboli G, Gardella E, Capovilla G. Idiopathic generalized epilepsy (IGE) syndromes in development: IGE with absences of early childhood, IGE with phantom absences, and perioral myoclonia with absences. Epilepsia 2009;50 (suppl 5):24–8. [2] d’Orsi G, Demaio V, Trivisano M, Pascarella MG, Specchio LM. Ictal videopolygraphic features of perioral myoclonia with absences. Epilepsy Behav 2011;21:314–7. [3] Vrielynck P, Rostomashvili N, Degroote E, Ghariani S, van Rijckevorsel K. Perioral myoclonia with absences and myoclonic status aggravated by oxcarbazepine. Epileptic Disord 2011;13:308–12. [4] Sevgi EB, Saygi S, Ciger A. Eye closure sensitivity and epileptic syndromes: a retrospective study of 26 adult cases. Seizure 2007;16:17–21. [5] Tekin Güveli B, Baykan B, Dörtcan N, Bebek N, Gürses C, Gökyig˘it A. Eye closure sensitivity in juvenile myoclonic epilepsy and its effect on prognosis. Seizure 2013;22:867–71. [6] Cappovilla G, Rubboli G, Beccaria F, et al. A clinical spectrum of the myoclonic manifestations associated with typical absences in childhood absence epilepsy. A video-polygraphic study. Epileptic Disord 2001;3:57–61. [7] Craiu D, Magureanu S, van Emde Boas W. Are absences truly generalized seizures or partial seizures originating from or predominantlyinvolving the premotor areas? Some clinical and theoretical observations and their implicationsfor seizure classification. Epilepsy Res 2006;70(suppl 1):S141–55. [8] Meeren H, van Luijtelaar G, Lopes da Silva F, Coenen A. Evolving concepts on the pathophysiology of absence seizures: the cortical focus theory. Arch Neurol 2005;62:371–6. [9] Rozendaal YJ, van Luijtelaar G, Ossenblok PP. Spatiotemporal mapping of interictal epileptiform discharges in human absence epilepsy: a MEG study. Epilepsy Res 2016;119:67–76.
https://doi.org/10.1016/j.jocn.2018.06.008
Effects of cognitive and motor rehabilitation in non-convulsive status epilepticus: A case report Francesco Corallo a,⇑, Viviana Lo Buono a, Marcella Di Cara a, Simona De Salvo a, Giuliana Vermiglio a, Patrizia Pollicino a, Placido Bramanti a, Silvia Marino a,b, Carmela Rifici a a b
IRCCS Centro Neurolesi ‘‘Bonino-Pulejo”, Messina, Italy Department of Biomedical and Dental Sciences and Morphological and Functional Imaging, University of Messina, Messina, Italy
a r t i c l e
i n f o
Article history: Received 7 February 2018 Accepted 6 June 2018
Keywords: Cognitive impairment Cognitive rehabilitation Non-convulsive status epilepticus
a b s t r a c t Epilepsy is a neurological disorder characterized by recurrent and unpredictable interruptions of normal brain function, that implies neurobiologic, psychological, and social consequences. Non-convulsive status epilepticus (NCSE) is an electro-clinical state associated to altered level of consciousness. In NCSE could occur the behavioural and emotional disorder, cognitive impairment and psychiatric disorder with a negative impact on quality of life and adaptive social behaviours. Cognitive rehabilitation for epileptic patients may include internal compensation strategies, external memory aids, psychoeducation, verbal and visual memory training, attention exercise and executive functions. We described the cognitive rehabilitation in a patient with NCSE characterized by an unusual cognitive impairment. A 55-year-old female patient, with epileptic seizures presented a severe impairment in cognitive function and focal neurological deficit. The cognitive treatment was designed as a combination of direct training of the impaired functions and metacognitive training to facilitate the development of compensatory strategies and it was divided into three methods. Neuropsychological evaluation showed a progressive deficit of high cognitive functions. The patient after cognitive rehabilitation, demonstrated a significant recovery of motor abilities, despite the neurocognitive profile was abnormal. Several advances have been made in this field but few studies on neuropsychological rehabilitation in adult patients with NCSE have been conducted and no standardized protocol or clinical guidelines are still available. Ó 2018 Elsevier Ltd. All rights reserved.
⇑ Corresponding author at: IRCCS Centro Neurolesi ‘‘Bonino-Pulejo”, S.S. 113 Via Palermo, C.da Casazza, 98124 Messina, Italy. E-mail address: [email protected] (F. Corallo).