Neurol Sci DOI 10.1007/s10072-013-1609-0
QUIZ CASES
Somatosensory reflex seizures in a patient with hypertrophic cranial pachymeningitis Rui-Juan Lv • Tao Cui • Yue-Tao Ma • Heng Zhou • Xing-Hu Zhang • Xiao-Qiu Shao
Received: 21 August 2013 / Accepted: 12 December 2013 Ó Springer-Verlag Italia 2013
Abstract Somatosensory-evoked reflex epilepsy is characterized by seizures in response to specific stimuli. It is highly uncommon for somatosensory-evoked focal seizures to be caused by movement or a change in posture. Reflex epilepsy induced by both somatosensory and proprioceptive stimulations has not been previously reported. In this study, we present a case of reflex epilepsy evoked by somatosensory and proprioceptive stimulation in a patient with hypertrophic cranial pachymeningitis. After comparing our patient with other cases of previously reported somatosensory-evoked reflex epilepsy, we determined that our patient had an unusual cause of reflex epilepsy.
evoked reflex epilepsy is evoked by somatic stimulation of a specific part of the body in the absence of either startle or surprise. Movement or a change in posture does not typically induce somatosensory-evoked focal seizures. In this report, we describe a patient with hypertrophic cranial pachymeningitis whose seizures could be evoked not only by a somatosensory touch (scratching the arch of his foot) but also by walking or by a large change in position.
Keywords Hypertrophic pachymeningitis Reflex epilepsy Somatosensory-evoked epilepsy Proprioceptive stimulation
A 29-year-old Chinese right-handed man suffered generalized tonic–clonic and simple partial seizures for 1 month. He initially had episodic bilateral lower-limb numbness followed by falling down with a generalized convulsion for approximately 1–2 min. He later presented with the same seizure twice. He had another seizure pattern characterized by right limb flexion and left upper limb extension followed by lower-limb bilateral jittering, predominantly the right lower limb, without loss of consciousness lasting for approximately 2 min. Surprisingly, this focal seizure could be evoked by scratching the arch of his foot, walking or a large change in position ([90° angle). The seizures induced in this patient were characterized by the following sequence: initially, paraesthesia in the middle or close to the trigger zone emerged, which spread to other body regions as a sensory Jacksonian march, followed by bilateral asymmetric posture tonic and clonic contractions. This sequence was repeated regularly. This type of evoked seizures occurred dozens of times per day. In addition, a refractory period followed each evoked seizure. This patient was born to healthy and non-consanguineous parents following an uneventful delivery. There was no
Introduction Reflex epilepsy is a type of epileptic syndrome determined by a specific precipitant stimulus and clinical-electroencephalography (EEG) response. The seizures of reflex epilepsy may be either generalized or focal. Specific precipitants commonly include visual, auditory, vestibular, and olfactory stimuli. Other types of stimuli include highlevel cognitive processes, emotional, and decision-making tasks. Among other complicated stimuli, somatosensory-
R.-J. Lv T. Cui Y.-T. Ma H. Zhou X.-H. Zhang X.-Q. Shao (&) Department of Neurology, Beijing Tiantan Hospital affiliated to Capital Medical University, 100050 Beijing, People’s Republic of China e-mail: [email protected]
Case report
123
Neurol Sci
Fig. 1 a Head computed tomography (CT) scans showing the falx and the tentorium as marked high density. b Axial T1-weighted magnetic resonance (MR) images showing the thickened falx and the dura as hypointense, with signal voids of the dilated cerebral veins over the cerebral cortex. c Axial T2-weighted MR images showing
the falx and the dura as markedly hypointense, with hyperintense areas in the bilateral parietal white matter. d Axial, coronal, and sagittal T1-weighted MR images with gadolinium showing meningeal enhancement over the cerebral cortex, with signals representing the dilated cerebral veins
family history of epilepsy. He underwent an operation 5 years ago because of left ear tympanitis. Moreover, he suffered from chronic headache for approximately 1 year. Upon admission, the positive finding of the neurological examination was neck stiffness for three fingers. All immunological tests, including the entire set of immune, thyroid function with thyroid antibodies, ANCA, ESR, ASO, and RF, were unremarkable. In addition, fluorescent treponemal antibody test was also negative in the serum and cerebrospinal fluid (CSF). The level of angiotensin-converting enzyme in the serum was within the normal limits. There were no abnormal findings for tuberculosis in the blood and CSF. The cranial pressure was [330 cmH2O. The white cell count was slightly elevated to 20 cells/mm3, and the protein level was moderately elevated to 95.4 mg/dl in the CSF. A chest radiography showed no abnormalities. Cranial computed tomography (CT) revealed a hyperdense dura mater, including the falx
and tentorium (Fig. 1a). T1-weighted magnetic resonance (MR) imaging yielded a thickened hypointense dura mater (Fig. 1b) and T2-weighted MR imaging showed ischemic lesions in the white matter of the bilateral parietal lobe, suggesting that the pial veins of the cerebral cortex were dilated (Fig. 1c). T1-weighted MR imaging with gadolinium showed the dura with intense enhancement (Fig. 1d). Interictal EEG was normal. EEG evolution is shown in Fig. 2 in each habitual seizure. The patient primarily manifested chronic headache and epilepsy, increased intracranial pressure, inflammatory CSF changes, and specific imaging findings, combined with a long history of left ear tympanitis. The presumptive diagnosis was inflammatory hypertrophic cranial pachymeningitis with venous infarction that was judged by venography. Anticonvulsants, including depakine (500 mg/day), phenobarbital (90 mg/day), and oxcarbazepine (600 mg/day), were administered to treat the focal seizures, and
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Neurol Sci
Fig. 2 Six separate 10-s epochs extracted from an ictal recording. a At seizure onset followed by a large change in position, the EEG showed periodic sharp waves over the central and parietal regions. b After 13 s, the rhythmic activity arising from the central and
parietal regions. c, d The rhythmic activity with evident propagation to all leads. e The generalization evolved to rhythmic activity from the central regions. f The EEG became normal
corticosteroids and antibiotics were used to inhibit the active inflammatory changes. The focal seizures decreased gradually and the somatosensory- and proprioceptive-evoked seizures greatly improve after 1 month of treatment. Written consent was obtained from this patient prior to the report.
patient evoked by scratching the arch of his foot was very similar to rub epilepsy, whereas this patient had bilateral asymmetric posture tonic seizures often caused by sensorimotor area lesion, which was different from the previous case reports of rub epilepsy [1, 2]. According to Kanemoto [2], rub epilepsy typically occurs in patients with no mental or neurological deficits and scalp EEG recordings have often been erroneously judged as normal. This patient did not display these characteristics because his seizure was secondary to cerebral lesions with an obviously abnormal EEG. In addition, our patient represented a case of partial
Discussion Somatosensory and proprioceptive stimulations both induce seizures separately. The seizure semiology of this
123
Neurol Sci
epilepsy with focal seizures induced not only by touch but also by walking and posture similar to that reported by Iriarte [3]. The mechanism involved in reflex epilepsy may arise from the complexity of the action that serves as the trigger. Walking and posture are both proprioceptive stimuli. It is well known that touch and proprioception possess the same neurotransmission pathway. This patient had bilateral parietal venous infarction including the sensorimotor area; therefore, he could present with a clinical seizure when there was either touch or proprioception stimuli afferent into the parietal lobes. Based on the above observation and test results, we conclude that the reflex epilepsy of this patient is more complex than somatosensory or proprioceptive reflex epilepsy. Hence, this is an unusual example of reflex epilepsy simultaneously induced by somatosensory and proprioceptive stimulation. Hypertrophic cranial pachymeningitis is an uncommon inflammatory disease; however, the frequency of detection has increased since the development of MR imaging. The common causes of this disease include infection, neoplasm, and autoimmune inflammation. This patient had a long history of left ear tympanitis, and foreign matter may have easily entered his brain through the ear passage. The CSF examination showed inflammatory change. In summary, infection is the most probable cause of seizures in this patient, although an occult malignant cause cannot be
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completely excluded. Follow-up imaging after 1 year of recovery was normal and also supported the presumptive diagnosis. Seizures caused by hypertrophic cranial pachymeningitis are notably common [4]; however, to the best of our knowledge, this report presents the first description of hypertrophic cranial pachymeningitis leading to somatosensory- and proprioceptive-evoked epilepsy. Acknowledgments The authors would like to thank the patient for participating in this study. This work was supported by the Natural Science Foundation of China (Grant 81301119) and a grant from the Youth Foundation of Beijing Tiantan Hospital (KY2011-14).
References 1. Hsieh CY, Chiou NJ, Wu YJ et al (2011) Somatosensory rub evoked reflex epilepsy of a temporal lobe origin. Neurol Sci 32(2):297–299 2. Kanemoto K, Watanabe Y, Tsuji T et al (2001) Rub epilepsy: a somatosensory evoked reflex epilepsy induced by prolonged cutaneous stimulation. J Neurol Neurosurg Psychiatry 70(4): 541–543 3. Iriarte J, Sanchez-Carpintero R, Schlumberger E et al (2001) Gait epilepsy. A case report of gait-induced seizures. Epilepsia 42(8):1087–1090 4. Tan K, Lim SA, Thomas A, Lim CT et al (2008) Idiopathic hypertrophic pachymeningitis causing seizures. Eur J Neurol 15(2):e12–e13
QUIZ CASES
Somatosensory reflex seizures in a patient with hypertrophic cranial pachymeningitis Rui-Juan Lv • Tao Cui • Yue-Tao Ma • Heng Zhou • Xing-Hu Zhang • Xiao-Qiu Shao
Received: 21 August 2013 / Accepted: 12 December 2013 Ó Springer-Verlag Italia 2013
Abstract Somatosensory-evoked reflex epilepsy is characterized by seizures in response to specific stimuli. It is highly uncommon for somatosensory-evoked focal seizures to be caused by movement or a change in posture. Reflex epilepsy induced by both somatosensory and proprioceptive stimulations has not been previously reported. In this study, we present a case of reflex epilepsy evoked by somatosensory and proprioceptive stimulation in a patient with hypertrophic cranial pachymeningitis. After comparing our patient with other cases of previously reported somatosensory-evoked reflex epilepsy, we determined that our patient had an unusual cause of reflex epilepsy.
evoked reflex epilepsy is evoked by somatic stimulation of a specific part of the body in the absence of either startle or surprise. Movement or a change in posture does not typically induce somatosensory-evoked focal seizures. In this report, we describe a patient with hypertrophic cranial pachymeningitis whose seizures could be evoked not only by a somatosensory touch (scratching the arch of his foot) but also by walking or by a large change in position.
Keywords Hypertrophic pachymeningitis Reflex epilepsy Somatosensory-evoked epilepsy Proprioceptive stimulation
A 29-year-old Chinese right-handed man suffered generalized tonic–clonic and simple partial seizures for 1 month. He initially had episodic bilateral lower-limb numbness followed by falling down with a generalized convulsion for approximately 1–2 min. He later presented with the same seizure twice. He had another seizure pattern characterized by right limb flexion and left upper limb extension followed by lower-limb bilateral jittering, predominantly the right lower limb, without loss of consciousness lasting for approximately 2 min. Surprisingly, this focal seizure could be evoked by scratching the arch of his foot, walking or a large change in position ([90° angle). The seizures induced in this patient were characterized by the following sequence: initially, paraesthesia in the middle or close to the trigger zone emerged, which spread to other body regions as a sensory Jacksonian march, followed by bilateral asymmetric posture tonic and clonic contractions. This sequence was repeated regularly. This type of evoked seizures occurred dozens of times per day. In addition, a refractory period followed each evoked seizure. This patient was born to healthy and non-consanguineous parents following an uneventful delivery. There was no
Introduction Reflex epilepsy is a type of epileptic syndrome determined by a specific precipitant stimulus and clinical-electroencephalography (EEG) response. The seizures of reflex epilepsy may be either generalized or focal. Specific precipitants commonly include visual, auditory, vestibular, and olfactory stimuli. Other types of stimuli include highlevel cognitive processes, emotional, and decision-making tasks. Among other complicated stimuli, somatosensory-
R.-J. Lv T. Cui Y.-T. Ma H. Zhou X.-H. Zhang X.-Q. Shao (&) Department of Neurology, Beijing Tiantan Hospital affiliated to Capital Medical University, 100050 Beijing, People’s Republic of China e-mail: [email protected]
Case report
123
Neurol Sci
Fig. 1 a Head computed tomography (CT) scans showing the falx and the tentorium as marked high density. b Axial T1-weighted magnetic resonance (MR) images showing the thickened falx and the dura as hypointense, with signal voids of the dilated cerebral veins over the cerebral cortex. c Axial T2-weighted MR images showing
the falx and the dura as markedly hypointense, with hyperintense areas in the bilateral parietal white matter. d Axial, coronal, and sagittal T1-weighted MR images with gadolinium showing meningeal enhancement over the cerebral cortex, with signals representing the dilated cerebral veins
family history of epilepsy. He underwent an operation 5 years ago because of left ear tympanitis. Moreover, he suffered from chronic headache for approximately 1 year. Upon admission, the positive finding of the neurological examination was neck stiffness for three fingers. All immunological tests, including the entire set of immune, thyroid function with thyroid antibodies, ANCA, ESR, ASO, and RF, were unremarkable. In addition, fluorescent treponemal antibody test was also negative in the serum and cerebrospinal fluid (CSF). The level of angiotensin-converting enzyme in the serum was within the normal limits. There were no abnormal findings for tuberculosis in the blood and CSF. The cranial pressure was [330 cmH2O. The white cell count was slightly elevated to 20 cells/mm3, and the protein level was moderately elevated to 95.4 mg/dl in the CSF. A chest radiography showed no abnormalities. Cranial computed tomography (CT) revealed a hyperdense dura mater, including the falx
and tentorium (Fig. 1a). T1-weighted magnetic resonance (MR) imaging yielded a thickened hypointense dura mater (Fig. 1b) and T2-weighted MR imaging showed ischemic lesions in the white matter of the bilateral parietal lobe, suggesting that the pial veins of the cerebral cortex were dilated (Fig. 1c). T1-weighted MR imaging with gadolinium showed the dura with intense enhancement (Fig. 1d). Interictal EEG was normal. EEG evolution is shown in Fig. 2 in each habitual seizure. The patient primarily manifested chronic headache and epilepsy, increased intracranial pressure, inflammatory CSF changes, and specific imaging findings, combined with a long history of left ear tympanitis. The presumptive diagnosis was inflammatory hypertrophic cranial pachymeningitis with venous infarction that was judged by venography. Anticonvulsants, including depakine (500 mg/day), phenobarbital (90 mg/day), and oxcarbazepine (600 mg/day), were administered to treat the focal seizures, and
123
Neurol Sci
Fig. 2 Six separate 10-s epochs extracted from an ictal recording. a At seizure onset followed by a large change in position, the EEG showed periodic sharp waves over the central and parietal regions. b After 13 s, the rhythmic activity arising from the central and
parietal regions. c, d The rhythmic activity with evident propagation to all leads. e The generalization evolved to rhythmic activity from the central regions. f The EEG became normal
corticosteroids and antibiotics were used to inhibit the active inflammatory changes. The focal seizures decreased gradually and the somatosensory- and proprioceptive-evoked seizures greatly improve after 1 month of treatment. Written consent was obtained from this patient prior to the report.
patient evoked by scratching the arch of his foot was very similar to rub epilepsy, whereas this patient had bilateral asymmetric posture tonic seizures often caused by sensorimotor area lesion, which was different from the previous case reports of rub epilepsy [1, 2]. According to Kanemoto [2], rub epilepsy typically occurs in patients with no mental or neurological deficits and scalp EEG recordings have often been erroneously judged as normal. This patient did not display these characteristics because his seizure was secondary to cerebral lesions with an obviously abnormal EEG. In addition, our patient represented a case of partial
Discussion Somatosensory and proprioceptive stimulations both induce seizures separately. The seizure semiology of this
123
Neurol Sci
epilepsy with focal seizures induced not only by touch but also by walking and posture similar to that reported by Iriarte [3]. The mechanism involved in reflex epilepsy may arise from the complexity of the action that serves as the trigger. Walking and posture are both proprioceptive stimuli. It is well known that touch and proprioception possess the same neurotransmission pathway. This patient had bilateral parietal venous infarction including the sensorimotor area; therefore, he could present with a clinical seizure when there was either touch or proprioception stimuli afferent into the parietal lobes. Based on the above observation and test results, we conclude that the reflex epilepsy of this patient is more complex than somatosensory or proprioceptive reflex epilepsy. Hence, this is an unusual example of reflex epilepsy simultaneously induced by somatosensory and proprioceptive stimulation. Hypertrophic cranial pachymeningitis is an uncommon inflammatory disease; however, the frequency of detection has increased since the development of MR imaging. The common causes of this disease include infection, neoplasm, and autoimmune inflammation. This patient had a long history of left ear tympanitis, and foreign matter may have easily entered his brain through the ear passage. The CSF examination showed inflammatory change. In summary, infection is the most probable cause of seizures in this patient, although an occult malignant cause cannot be
123
completely excluded. Follow-up imaging after 1 year of recovery was normal and also supported the presumptive diagnosis. Seizures caused by hypertrophic cranial pachymeningitis are notably common [4]; however, to the best of our knowledge, this report presents the first description of hypertrophic cranial pachymeningitis leading to somatosensory- and proprioceptive-evoked epilepsy. Acknowledgments The authors would like to thank the patient for participating in this study. This work was supported by the Natural Science Foundation of China (Grant 81301119) and a grant from the Youth Foundation of Beijing Tiantan Hospital (KY2011-14).
References 1. Hsieh CY, Chiou NJ, Wu YJ et al (2011) Somatosensory rub evoked reflex epilepsy of a temporal lobe origin. Neurol Sci 32(2):297–299 2. Kanemoto K, Watanabe Y, Tsuji T et al (2001) Rub epilepsy: a somatosensory evoked reflex epilepsy induced by prolonged cutaneous stimulation. J Neurol Neurosurg Psychiatry 70(4): 541–543 3. Iriarte J, Sanchez-Carpintero R, Schlumberger E et al (2001) Gait epilepsy. A case report of gait-induced seizures. Epilepsia 42(8):1087–1090 4. Tan K, Lim SA, Thomas A, Lim CT et al (2008) Idiopathic hypertrophic pachymeningitis causing seizures. Eur J Neurol 15(2):e12–e13
