- Email: [email protected]
Complex partial status epilepticus associated with adult H1N1 infection
1728
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1728–1730
Our patient’s Marfan’s syndrome, with ligamentous laxity, was responsible for the development of his BI and CM. Avoiding posterior fossa decompression in this instance provided maximal bone surface for osteointegration of the OC arthrodesis and minimized the potential for additional surgical morbidity. If the syrinx had not resolved, he would have required additional surgery to decompress the malformation, to drain the syrinx with a myelotomy, or both. We report a cervical syrinx that resolved in a patient with circumferential compression of the foramen magnum with ventral transoral decompression of the foramen magnum. Posterior OC occipitocervical fusion was performed without the need for posterior decompression of the CM. References
Fig. 3. Three-month follow-up sagittal T2-weighted MRI confirming complete resolution of the cervical syrinx and showing no compression at the foramen magnum. Used with permission from Barrow Neurological Institute.
tively, the spinal instrumentation precluded postoperative MRI to confirm resolution of the syrinx.
1. Heiss JD, Patronas N, DeVroom HL, et al. Elucidating the pathophysiology of syringomyelia. J Neurosurg 1999;91:553–62. 2. Hida K, Iwasaki Y. Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Neurosurg Focus 2001;11:E7. 3. Lund-Johansen M, Wester K. Syringomyelia treated with a nonvalved syringoperitoneal shunt: a follow-up study. Neurosurgery 1997;41:858–64. 4. Park TS, Cail WS, Broaddus WC, et al. Lumboperitoneal shunt combined with myelotomy for treatment of syringohydromyelia. J Neurosurg 1989;70:721–7. 5. Grabb PA, Mapstone TB, Oakes WJ. Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery 1999; 44:520–7. 6. Peruzzotti-Jametti L, Ferrari S, Politi LS, et al. Giant anterior arachnoid cyst associated with syringomyelia: implications for treatment and follow-up. Spine (Phila Pa 1976) 2010;35:E322–4. 7. Sawin PD, Menezes AH. Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management. J Neurosurg 1997;86:950–60. 8. Goel A. Treatment of basilar invagination by atlantoaxial joint distraction and direct lateral mass fixation. J Neurosurg Spine 2004;1:281–6. 9. Goel A, Bhatjiwale M, Desai K. Basilar invagination: a study based on 190 surgically treated patients. J Neurosurg 1998;88:962–8. 10. Goel A, Shah A. Reversal of longstanding musculoskeletal changes in basilar invagination after surgical decompression and stabilization. J Neurosurg Spine 2009;10:220–7.
doi:http://dx.doi.org/10.1016/j.jocn.2012.01.037
Complex partial status epilepticus associated with adult H1N1 infection L.L.L. Yeo ⇑, P.R. Paliwal, P.A. Tambyah, D.P. Olszyna, E. Wilder-Smith, R. Rathakrishnan Department of Neurology, National University Hospital, 5 Lower Kent Ridge Road, Singapore 119074, Singapore
a r t i c l e
i n f o
Article history: Received 31 December 2011 Accepted 4 January 2012
Keywords: Epilepsy H1N1 Infectious disease Status epilpeticus
a b s t r a c t In the wake of the worldwide H1N1 pandemic, there has been evidence that the H1N1 influenza virus is associated with neurological complications. This is the first report describing status epilepticus in an adult patient with H1N1 virus infection, to our knowledge. This patient had no prior history of epilepsy and presented with complex partial status epilepticus. This was further illustrated on electroencephalographs and MRI brain changes that corresponded with the patient’s clinical state and which subsequently resolved on follow-up. Although uncommon, H1N1 infections may result in central nervous system complications in adults and it is crucial to treat such patients with urgency. Ó 2012 Elsevier Ltd. All rights reserved.
1. Case report A 42-year-old woman presented to the emergency department with light-headedness and subsequently lost consciousness for 5
⇑ Corresponding author. Tel.: +65 67722516. E-mail address: [email protected] (L.L.L. Yeo).
minutes. The event was witnessed by her husband who noticed persistent up rolling of the eyes without any convulsions. Two days prior to admission, she complained of fever and upper respiratory tract symptoms. In the emergency room, she was drowsy but easily rousable. There were no signs of meningeal irritation and no focal neurological deficits. As this was during the H1N1 pandemic, and in view of her upper respiratory tract symptoms, a nasopharyngeal swab was
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1728–1730
1729
Fig. 1. (A–C) Electroencephalographs (EEG) and (D–I) MRI of an adult patient with complex partial status epilepticus associated with H1N1 infection showing (A) periodic discharges arising from the right posterior quadrant, also involving the left posterior temporal and occipital regions, (B) resolution of ictal activity after an infusion of intravenous valproate (25 mg/kg), and (C) 3-months later, a return of normal background alpha rhythm on repeat EEG. (D–F) Axial T2-weighted (D) and coronal fluid attenuated inversion recovery (FLAIR, E, F) MRI showing T2-weighted cortical and subcortical white matter hyperintensities without restricted diffusion in both parietooccipital and frontal regions, and (G–I) Axial T2-weighted (G) and coronal FLAIR (H, I) MRI 3 months later showing resolution of T2-weighted hyperintensities (This figure is available in colour at www.sciencedirect.com.).
sent for gel-based polymerase chain reaction (PCR) for influenza A, H1, H3, H5, N1, and N2. This swab was found to be positive, indicating a H1N1 infection. Probe-based influenza (H1N1) 2009 PCR and partial sequencing of the matrix gene was conducted to confirm the result.1 In keeping with public health policy at the time, the patient was isolated and commenced on a course of the antiviral oseltamivir. Two days into her hospital stay, she developed three generalised tonic–clonic seizures. The first two seizures aborted spontaneously after 2 minutes while the third seizure lasted for 5 minutes and was terminated with 2 mg of lorazapam. All three seizures occurred over a period of 20 minutes and consciousness was not regained between seizures. Her blood sugar level was 14 mmol/L at the time and her electrolytes were within the normal range. The patient was treated with 20 mg/kg of phenytoin. During this period there was persistent deviation of her eyes to the left and facial myoclonus. The patient did not respond to verbal commands. The electroencephalogram (EEG) showed periodic discharges arising from the right posterior quadrant, also involving the left posterior temporal and occipital regions (Fig. 1A). An infusion of intravenous valproate at 25 mg/kg was commenced with resolution of both clinical and EEG ictal activity (Fig. 1B). There was no evidence of metabolic causes of seizures or electrolyte imbalance. Contrast-enhanced brain MRI showed T2-weighted cortical and subcortical white matter hyperintensities without restricted diffu-
sion in both parieto-occipital and frontal regions (Fig. 1D–F). Cerebrospinal fluid (CSF) analysis showed 3 white cells and an elevated protein concentration of 3 g/L. The CSF PCR for H1N1 was negative, as were bacterial cultures and viral serology. Within 48 hours, the patient made a complete recovery and remained free of seizures at her 12-month follow-up appointment. MRI brain repeated 3 months later showed resolution of T2weighted hyperintensities (Fig. 1G–I). A repeat EEG showed a return of normal background alpha rhythm (Fig. 1C).
2. Discussion The H1N1 virus in adults is rarely associated with seizures or alteration of consciousness level.2 The majority of influenza-related neurological complications occur in the paediatric population. To our knowledge, this is the first report describing status epilepticus in an adult with H1N1 virus infection.3 Neuroimaging in severe paediatric influenza-associated encephalopathy may show diffuse cerebral oedema and bilateral thalamic lesions.3 In our adult patient the lesions were seen in the parieto-occipital and frontal regions instead and they had resolved on follow-up. The difference in distribution may be reflective of a preferential site of involvement in the adult brain, similar to the predilection for the herpes simplex virus to affect the temporal lobes, although seizure activity may possibly result
1730
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1730–1733
in these changes as well and it is difficult to identify their exact aetiology. The EEG in paediatric patients with influenza-associated encephalopathy has been reported to show diffuse abnormalities.3 In our patient the EEG showed bilateral temporal rhythmic sharpwave discharges < 2.5/s with focal ictal phenomena of facial twitching and eye deviation in keeping with a clinico-electrographical status epilepticus.4 The influenza virus is very rarely isolated in the CSF; detection of viral antigen or genome in CSF is also uncommon. Even in autopsied individuals, influenza virus or genetic material is hardly ever isolated from brain tissue.5 In our patient the HINI virus was not isolated from the CSF. Antiviral medication reduces the risk of complications from influenza virus infections; thus, antiviral treatment should be started as quickly as possible.6 As the test often takes time to process, it is prudent to start empirical therapy before the final result. It is highly unlikely that the antiviral medication in our patient triggered the status epilepticus. In a study of 40,000 patients of all ages diagnosed with influenza and prescribed oseltamivir, the number of central nervous system (CNS)-related and neuropsychiatric episodes was fewer in the group taking oseltamivir.7
3. Conclusion Whereas H1N1-related status epilepticus appears to be rare considering the number of infected patients in the recent pandemic, we should be vigilant nonetheless with regard to CNS complications. Antiviral medication reduces the risk of complications from influenza viral infections and should be started as quickly as possible based on clinical suspicion.6 References 1. Leo YS, Lye DC, Barkham T, et al. Pandemic (H1N1) 2009 surveillance and prevalence of seasonal influenza, Singapore. Emerg Infect Dis 2010;16:103–5. 2. Kitcharoen S, Pattapongsin M, Sawanyawisuth K, et al. Neurologic manifestations of pandemic (H1N1) 2009 virus infection. Emerg Infect Dis 2010 Mar;16:569–70. 3. Amin R, Ford-Jones E, Richardson SE, et al. Acute childhood encephalitis and encephalopathy associated with influenza: a prospective 11-year review. Pediatr Infect Dis J 2008;27:390–5. 4. Kaplan PW. EEG criteria for nonconvulsive status epilepticus. Epilepsia 2007;48:39–41. 5. Morishima T, Togashi T, Yokota S, et al. Encephalitis and encephalopathy associated with an influenza epidemic in Japan. Clin Infect Dis 2002;35:512–7. 6. Kaiser L, Wat C, Mills T, et al. Impact of oseltamivir treatment on influenzarelated lower respiratory tract complications and hospitalizations. Arch Intern Med 2003;163:1667–72. 7. Blumentals WA, Song X. The safety of oseltamivir in patients with influenza: analysis of healthcare claims data from six influenza seasons. MedGenMed 2007;9:23.
doi:http://dx.doi.org/10.1016/j.jocn.2012.01.037
Capsular warning syndrome and crescendo lacunar strokes after atherosclerotic stenosis of the recurrent artery of Heubner José E. Cohen a,b,⇑, Alejandro Rabinstein c, John M. Gomori b, Ronen R. Leker d a
Department of Neurosurgery, Hadassah University Medical Center, Ein-Kerem, P.O. Box 12,000, Jerusalem 91120, Israel Department of Radiology, Hadassah–Hebrew University Medical Center, Jerusalem, Israel c Department of Neurology, Mayo Clinics, Rochester, Minnesota, USA d Department of Neurology, Hadassah–Hebrew University Medical Center, Jerusalem, Israel b
a r t i c l e
i n f o
Article history: Received 18 April 2012 Accepted 22 April 2012
Keywords: Capsular warning syndrome Capsular stroke Heubner disease Lacunar stroke Stroke mechanisms
a b s t r a c t The stereotype of repetitive transient cerebral ischemia causing unilateral motor, sensory, or sensorimotor deficits that simultaneously affect the face, arm, and leg, clinically localized to the internal capsule, fits with the description of capsular warning syndrome (CWS). A high proportion of individuals with these symptoms develop subsequent capsular stroke, despite various proposed preventative measures. It has been postulated that the mechanism for such strokes is that of small-vessel single-penetrator disease. We present a patient with repetitive CWS intermingled with crescendo capsular strokes secondary to recurrent artery of Heubner disease. This report causally links CWS–crescendo lacunar strokes and Heubner artery atherosclerotic disease (intracranial branch atheromatous disease). Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction Little is known about stroke mechanisms or the role of branch atherosclerotic disease in patients with fluctuating symptoms. The stereotypic nature of repeated symptoms clinically localized
⇑ Corresponding author. Tel.: +972 2 6777092. E-mail address: [email protected] (J.E. Cohen).
to the internal capsule fits with the description of capsular warning syndrome (CWS).1,2 A high proportion of individuals presenting with these symptoms develop subsequent capsular stroke.2 It has been postulated that the mechanism for such strokes is that of small-vessel single-penetrator disease. We present a patient with repetitive CWS followed by capsular strokes secondary to recurrent artery of Heubner (RAH) disease. The RAH presented a combination of ostial stenosis and distal occlusion. We propose that ostial stenosis causes distal artery hypoperfusion leading to progressive occlusion of the vessel in a distal-to-proximal direction
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1728–1730
Our patient’s Marfan’s syndrome, with ligamentous laxity, was responsible for the development of his BI and CM. Avoiding posterior fossa decompression in this instance provided maximal bone surface for osteointegration of the OC arthrodesis and minimized the potential for additional surgical morbidity. If the syrinx had not resolved, he would have required additional surgery to decompress the malformation, to drain the syrinx with a myelotomy, or both. We report a cervical syrinx that resolved in a patient with circumferential compression of the foramen magnum with ventral transoral decompression of the foramen magnum. Posterior OC occipitocervical fusion was performed without the need for posterior decompression of the CM. References
Fig. 3. Three-month follow-up sagittal T2-weighted MRI confirming complete resolution of the cervical syrinx and showing no compression at the foramen magnum. Used with permission from Barrow Neurological Institute.
tively, the spinal instrumentation precluded postoperative MRI to confirm resolution of the syrinx.
1. Heiss JD, Patronas N, DeVroom HL, et al. Elucidating the pathophysiology of syringomyelia. J Neurosurg 1999;91:553–62. 2. Hida K, Iwasaki Y. Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation. Neurosurg Focus 2001;11:E7. 3. Lund-Johansen M, Wester K. Syringomyelia treated with a nonvalved syringoperitoneal shunt: a follow-up study. Neurosurgery 1997;41:858–64. 4. Park TS, Cail WS, Broaddus WC, et al. Lumboperitoneal shunt combined with myelotomy for treatment of syringohydromyelia. J Neurosurg 1989;70:721–7. 5. Grabb PA, Mapstone TB, Oakes WJ. Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery 1999; 44:520–7. 6. Peruzzotti-Jametti L, Ferrari S, Politi LS, et al. Giant anterior arachnoid cyst associated with syringomyelia: implications for treatment and follow-up. Spine (Phila Pa 1976) 2010;35:E322–4. 7. Sawin PD, Menezes AH. Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management. J Neurosurg 1997;86:950–60. 8. Goel A. Treatment of basilar invagination by atlantoaxial joint distraction and direct lateral mass fixation. J Neurosurg Spine 2004;1:281–6. 9. Goel A, Bhatjiwale M, Desai K. Basilar invagination: a study based on 190 surgically treated patients. J Neurosurg 1998;88:962–8. 10. Goel A, Shah A. Reversal of longstanding musculoskeletal changes in basilar invagination after surgical decompression and stabilization. J Neurosurg Spine 2009;10:220–7.
doi:http://dx.doi.org/10.1016/j.jocn.2012.01.037
Complex partial status epilepticus associated with adult H1N1 infection L.L.L. Yeo ⇑, P.R. Paliwal, P.A. Tambyah, D.P. Olszyna, E. Wilder-Smith, R. Rathakrishnan Department of Neurology, National University Hospital, 5 Lower Kent Ridge Road, Singapore 119074, Singapore
a r t i c l e
i n f o
Article history: Received 31 December 2011 Accepted 4 January 2012
Keywords: Epilepsy H1N1 Infectious disease Status epilpeticus
a b s t r a c t In the wake of the worldwide H1N1 pandemic, there has been evidence that the H1N1 influenza virus is associated with neurological complications. This is the first report describing status epilepticus in an adult patient with H1N1 virus infection, to our knowledge. This patient had no prior history of epilepsy and presented with complex partial status epilepticus. This was further illustrated on electroencephalographs and MRI brain changes that corresponded with the patient’s clinical state and which subsequently resolved on follow-up. Although uncommon, H1N1 infections may result in central nervous system complications in adults and it is crucial to treat such patients with urgency. Ó 2012 Elsevier Ltd. All rights reserved.
1. Case report A 42-year-old woman presented to the emergency department with light-headedness and subsequently lost consciousness for 5
⇑ Corresponding author. Tel.: +65 67722516. E-mail address: [email protected] (L.L.L. Yeo).
minutes. The event was witnessed by her husband who noticed persistent up rolling of the eyes without any convulsions. Two days prior to admission, she complained of fever and upper respiratory tract symptoms. In the emergency room, she was drowsy but easily rousable. There were no signs of meningeal irritation and no focal neurological deficits. As this was during the H1N1 pandemic, and in view of her upper respiratory tract symptoms, a nasopharyngeal swab was
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1728–1730
1729
Fig. 1. (A–C) Electroencephalographs (EEG) and (D–I) MRI of an adult patient with complex partial status epilepticus associated with H1N1 infection showing (A) periodic discharges arising from the right posterior quadrant, also involving the left posterior temporal and occipital regions, (B) resolution of ictal activity after an infusion of intravenous valproate (25 mg/kg), and (C) 3-months later, a return of normal background alpha rhythm on repeat EEG. (D–F) Axial T2-weighted (D) and coronal fluid attenuated inversion recovery (FLAIR, E, F) MRI showing T2-weighted cortical and subcortical white matter hyperintensities without restricted diffusion in both parietooccipital and frontal regions, and (G–I) Axial T2-weighted (G) and coronal FLAIR (H, I) MRI 3 months later showing resolution of T2-weighted hyperintensities (This figure is available in colour at www.sciencedirect.com.).
sent for gel-based polymerase chain reaction (PCR) for influenza A, H1, H3, H5, N1, and N2. This swab was found to be positive, indicating a H1N1 infection. Probe-based influenza (H1N1) 2009 PCR and partial sequencing of the matrix gene was conducted to confirm the result.1 In keeping with public health policy at the time, the patient was isolated and commenced on a course of the antiviral oseltamivir. Two days into her hospital stay, she developed three generalised tonic–clonic seizures. The first two seizures aborted spontaneously after 2 minutes while the third seizure lasted for 5 minutes and was terminated with 2 mg of lorazapam. All three seizures occurred over a period of 20 minutes and consciousness was not regained between seizures. Her blood sugar level was 14 mmol/L at the time and her electrolytes were within the normal range. The patient was treated with 20 mg/kg of phenytoin. During this period there was persistent deviation of her eyes to the left and facial myoclonus. The patient did not respond to verbal commands. The electroencephalogram (EEG) showed periodic discharges arising from the right posterior quadrant, also involving the left posterior temporal and occipital regions (Fig. 1A). An infusion of intravenous valproate at 25 mg/kg was commenced with resolution of both clinical and EEG ictal activity (Fig. 1B). There was no evidence of metabolic causes of seizures or electrolyte imbalance. Contrast-enhanced brain MRI showed T2-weighted cortical and subcortical white matter hyperintensities without restricted diffu-
sion in both parieto-occipital and frontal regions (Fig. 1D–F). Cerebrospinal fluid (CSF) analysis showed 3 white cells and an elevated protein concentration of 3 g/L. The CSF PCR for H1N1 was negative, as were bacterial cultures and viral serology. Within 48 hours, the patient made a complete recovery and remained free of seizures at her 12-month follow-up appointment. MRI brain repeated 3 months later showed resolution of T2weighted hyperintensities (Fig. 1G–I). A repeat EEG showed a return of normal background alpha rhythm (Fig. 1C).
2. Discussion The H1N1 virus in adults is rarely associated with seizures or alteration of consciousness level.2 The majority of influenza-related neurological complications occur in the paediatric population. To our knowledge, this is the first report describing status epilepticus in an adult with H1N1 virus infection.3 Neuroimaging in severe paediatric influenza-associated encephalopathy may show diffuse cerebral oedema and bilateral thalamic lesions.3 In our adult patient the lesions were seen in the parieto-occipital and frontal regions instead and they had resolved on follow-up. The difference in distribution may be reflective of a preferential site of involvement in the adult brain, similar to the predilection for the herpes simplex virus to affect the temporal lobes, although seizure activity may possibly result
1730
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1730–1733
in these changes as well and it is difficult to identify their exact aetiology. The EEG in paediatric patients with influenza-associated encephalopathy has been reported to show diffuse abnormalities.3 In our patient the EEG showed bilateral temporal rhythmic sharpwave discharges < 2.5/s with focal ictal phenomena of facial twitching and eye deviation in keeping with a clinico-electrographical status epilepticus.4 The influenza virus is very rarely isolated in the CSF; detection of viral antigen or genome in CSF is also uncommon. Even in autopsied individuals, influenza virus or genetic material is hardly ever isolated from brain tissue.5 In our patient the HINI virus was not isolated from the CSF. Antiviral medication reduces the risk of complications from influenza virus infections; thus, antiviral treatment should be started as quickly as possible.6 As the test often takes time to process, it is prudent to start empirical therapy before the final result. It is highly unlikely that the antiviral medication in our patient triggered the status epilepticus. In a study of 40,000 patients of all ages diagnosed with influenza and prescribed oseltamivir, the number of central nervous system (CNS)-related and neuropsychiatric episodes was fewer in the group taking oseltamivir.7
3. Conclusion Whereas H1N1-related status epilepticus appears to be rare considering the number of infected patients in the recent pandemic, we should be vigilant nonetheless with regard to CNS complications. Antiviral medication reduces the risk of complications from influenza viral infections and should be started as quickly as possible based on clinical suspicion.6 References 1. Leo YS, Lye DC, Barkham T, et al. Pandemic (H1N1) 2009 surveillance and prevalence of seasonal influenza, Singapore. Emerg Infect Dis 2010;16:103–5. 2. Kitcharoen S, Pattapongsin M, Sawanyawisuth K, et al. Neurologic manifestations of pandemic (H1N1) 2009 virus infection. Emerg Infect Dis 2010 Mar;16:569–70. 3. Amin R, Ford-Jones E, Richardson SE, et al. Acute childhood encephalitis and encephalopathy associated with influenza: a prospective 11-year review. Pediatr Infect Dis J 2008;27:390–5. 4. Kaplan PW. EEG criteria for nonconvulsive status epilepticus. Epilepsia 2007;48:39–41. 5. Morishima T, Togashi T, Yokota S, et al. Encephalitis and encephalopathy associated with an influenza epidemic in Japan. Clin Infect Dis 2002;35:512–7. 6. Kaiser L, Wat C, Mills T, et al. Impact of oseltamivir treatment on influenzarelated lower respiratory tract complications and hospitalizations. Arch Intern Med 2003;163:1667–72. 7. Blumentals WA, Song X. The safety of oseltamivir in patients with influenza: analysis of healthcare claims data from six influenza seasons. MedGenMed 2007;9:23.
doi:http://dx.doi.org/10.1016/j.jocn.2012.01.037
Capsular warning syndrome and crescendo lacunar strokes after atherosclerotic stenosis of the recurrent artery of Heubner José E. Cohen a,b,⇑, Alejandro Rabinstein c, John M. Gomori b, Ronen R. Leker d a
Department of Neurosurgery, Hadassah University Medical Center, Ein-Kerem, P.O. Box 12,000, Jerusalem 91120, Israel Department of Radiology, Hadassah–Hebrew University Medical Center, Jerusalem, Israel c Department of Neurology, Mayo Clinics, Rochester, Minnesota, USA d Department of Neurology, Hadassah–Hebrew University Medical Center, Jerusalem, Israel b
a r t i c l e
i n f o
Article history: Received 18 April 2012 Accepted 22 April 2012
Keywords: Capsular warning syndrome Capsular stroke Heubner disease Lacunar stroke Stroke mechanisms
a b s t r a c t The stereotype of repetitive transient cerebral ischemia causing unilateral motor, sensory, or sensorimotor deficits that simultaneously affect the face, arm, and leg, clinically localized to the internal capsule, fits with the description of capsular warning syndrome (CWS). A high proportion of individuals with these symptoms develop subsequent capsular stroke, despite various proposed preventative measures. It has been postulated that the mechanism for such strokes is that of small-vessel single-penetrator disease. We present a patient with repetitive CWS intermingled with crescendo capsular strokes secondary to recurrent artery of Heubner disease. This report causally links CWS–crescendo lacunar strokes and Heubner artery atherosclerotic disease (intracranial branch atheromatous disease). Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction Little is known about stroke mechanisms or the role of branch atherosclerotic disease in patients with fluctuating symptoms. The stereotypic nature of repeated symptoms clinically localized
⇑ Corresponding author. Tel.: +972 2 6777092. E-mail address: [email protected] (J.E. Cohen).
to the internal capsule fits with the description of capsular warning syndrome (CWS).1,2 A high proportion of individuals presenting with these symptoms develop subsequent capsular stroke.2 It has been postulated that the mechanism for such strokes is that of small-vessel single-penetrator disease. We present a patient with repetitive CWS followed by capsular strokes secondary to recurrent artery of Heubner (RAH) disease. The RAH presented a combination of ostial stenosis and distal occlusion. We propose that ostial stenosis causes distal artery hypoperfusion leading to progressive occlusion of the vessel in a distal-to-proximal direction