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A new MR radiological sign in HaNDL syndrome. A case report
Journal of Clinical Neuroscience xxx (xxxx) xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
A new MR radiological sign in HaNDL syndrome. A case report Claudia Rodríguez-López a,⇑,1, Nicolás Garzo Caldas a,1, David Uriarte Pérez de Urabayen a, Mario Sánchez Tornero a, Amaya Hilario Barrio b, Rosana Saiz Díaz a, Jesús González de la Aleja a a b
Department of Neurology, 12 de Octubre University Hospital, Madrid, Spain Department of Neuroradiology, 12 de Octubre University Hospital, Madrid, Spain
a r t i c l e
i n f o
Article history: Received 18 July 2018 Accepted 5 November 2018 Available online xxxx Keywords: HaNDL syndrome Susceptibility weighed MR Prominence vessel sign Oxygen extraction fraction
a b s t r a c t Background: One of the classical diagnostic criteria of the syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL syndrome) is the normality of imaging studies except from some reversible alterations as leptomeningeal enhancement or focal hypoperfusion. Case: We present a 41 year-old man who abruptly started with a set of right parietal symptoms, meeting the diagnostic criteria for HaNDL syndrome. An electroencephalographic record showed a slowing of the right hemisphere. MR susceptibility weighted sequences demonstrated a reduced venous signal in the symptomatic hemisphere, unlike other transient disorders as migraine aura where an opposite pattern with prominence of the venous structures in the symptomatic hemisphere has been reported. This sign could reflect a decrease in metabolic demands or a fail in oxygen employment by the affected tissue. Conclusion: To our knowledge, this finding has not been described yet, and it may provide a new insight on the pathogenesis of HaNDL syndrome. Ó 2018 Elsevier Ltd. All rights reserved.
1. Introduction The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL syndrome) [1] remains as a disorder of unknown origin and a poorly understood pathogenesis. One of its classical diagnostic criteria is the normality of all the imaging studies; nevertheless, some reversible alterations have been recently described. We present a case of HaNDL syndrome with new and unique radiological findings. 2. Case report A 41 years-old man without vascular risk factors nor antecedents of migraine presented with sudden, intense headache, right hemiparesis and motor aphasia which resolved spontaneously after 80 min. He was previously diagnosed of transient ischemic attack in another hospital and was referred to a stroke prevention clinic. Three days later he developed an identical set of symptoms lasting 60 min and after a period of three hours free from focality, he started with inattention, left central facial paresis, left hemianopia and left alien limb. This situation was associated again with striking headache as well as fever (38 °C). During the symptomatic ⇑ Corresponding author at: Department of Neurology, 12 de Octubre University Hospital, Avenida de Córdoba S/N, 28041 Madrid, Spain. E-mail address: [email protected] (C. Rodríguez-López). 1 Both authors contributed equally.
period the following studies were performed: firstly, a basal CT showed no alterations and an angioCT excluded intracranial occlusions. MR discarded restricted diffusion and revealed leptomeningeal enhancement in FLAIR sequences with contrast (Fig. 1A) as well as decreased prominence of the venous vasculature within the right hemisphere (Fig. 1B) in susceptibility weighted imaging (SWI). Finally, a lumbar puncture showed sterile lymphocytic pleocytosis (40 cells, 90% mononuclears) with hyperproteinorrhaquia (1.86 g/L), and the electroencephalogram demonstrated an asymmetrical diffuse slowing, more pronounced in the right hemisphere (Fig. 2A). Laboratory workup was normal. Extensive serum and CSF microbiological tests were negative. He was diagnosed of HaNDL syndrome according to the International Classification of Headache Disorders, 3rd edition (ICHD-3) [2]. His symptoms completely solved after 48 h, parallel to electroencephalogram normalization (Fig. 2B), without any specific treatment but analgesics. Eight days later he experimented once again right hemiparesis and motor aphasia in the set of intense headache with autolimitation in 60 min, and he was discharged with corticosteroid treatment in descending regimen. He has remained asymptomatic during a year follow-up.
3. Discussion Neuroimaging in HaNDL syndrome is usually normal; however some nonspecific abnormalities have been reported, such as mild
https://doi.org/10.1016/j.jocn.2018.11.018 0967-5868/Ó 2018 Elsevier Ltd. All rights reserved.
Please cite this article as: C. Rodríguez-López, N. Garzo Caldas, D. Uriarte Pérez de Urabayen et al., A new MR radiological sign in HaNDL syndrome. A case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2018.11.018
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Case report / Journal of Clinical Neuroscience xxx (xxxx) xxx
Fig. 1. Axial MR images during the symptomatic right parietal syndrome. (A) FLAIR sequence with contrast revealed leptomeningeal enhancement at different levels. (B) SWI showed a normal prominence of venous structures within the left hemisphere but a reduced prominence of the venous vasculature within the right one at different levels.
Fig. 2. Electroencephalographic records of the patient. (A) During the symptomatic right parietal syndrome an asymmetrical persistent bilateral slowing was identified. A poorly organized background can be appreciated, as well as irregular and diffuse theta activity intermixed with delta activity, being more pronounced on the right hemisphere. (B) One day after remaining asymptomatic, electroencephalographic normalization was demonstrated.
Please cite this article as: C. Rodríguez-López, N. Garzo Caldas, D. Uriarte Pérez de Urabayen et al., A new MR radiological sign in HaNDL syndrome. A case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2018.11.018
Case report / Journal of Clinical Neuroscience xxx (xxxx) xxx
leptomeningeal enhancement [3,4] or focal regions of hypoperfusion that correlated with the neurological deficits [5–7]. In our case, no perfusion techniques were performed. Nevertheless, a SWI was carried out in search of alterations, since prominence of the venous vasculature (prominence vessel sign, PVS) within the symptomatic brain region has been reported in other entities, such as migraine aura [8] or Todd palsy [9]. The PVS in the draining veins within areas of impaired perfusion has also been described in ischemic stroke [10], and it has been interpreted as the uncoupling between oxygen supply and demand in hypoperfused tissue, which would cause a relative increase of deoxyhemoglobin levels and a decrease of oxyhemoglobin in the tissue capillaries and the draining veins [11]. Surprisingly, our patient presented an opposite pattern, with reduced venous signal in the symptomatic hemisphere where we also observed a focal electroencephalographic slowing. This reduced venous signal, possibly caused by the decreased oxygen extraction fraction, could indicate a decrease in metabolic demands or a fail in oxygen employment by the affected tissue. Thus, the mechanism responsible for the deficits in HaNDL syndrome could be different to that known in migraine aura or postictal neurological deficits. In any case, given the unknown etiology and pathophysiology of HaNDL syndrome, this finding raises a number of interesting questions. Finally, we would like to remark the atypical nature of the symptoms of this patient, since the characteristic manifestations of HaNDL syndrome are hemimotor, hemisensory and aphasic deficits [12], but an isolated parietal syndrome has not been described to date in this entity to our knowledge. 4. Conclusion If confirmed, the reduced venous signal within the symptomatic region seen in SWI could represent an useful clinical tool in the diagnosis of HaNDL syndrome, since it has not been described in any other else to our knowledge, and it could suggest a different pathophysiology underlying HaNDL syndrome.
3
Acknowledgements This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. The authors declare that there is no conflict of interest. References [1] Berg MJ, Williams LS. The transient syndrome of headache with neurologic deficits and CSF lymphocytosis. Neurology 1995;45:1648–54. [2] Headache Classification Committee of the International Headache Society (IHS). The International classification of headache disorders, 3rd edition. Cephalalgia 2013;33:629–808. [3] Emond H, Schnorf H, Poloni C, Vulliemoz S, Lalive PH. Syndrome of transient headache and neurological deficits with CSF lymphocytosis (HaNDL) associated with recent human herpesvirus-6 infection. Cephalalgia 2009;29:487–91. [4] Patel N, Gupta V, Barrett K, Vibhute P. A syndrome of headache and neurological deficits with cerebrospinal fluid (CSF) lymphocytic pleocytosis (Handl) with diffuse vasogenic leakage of gadolinium MRI contrast into the extra-axial CSF cisterns. J Radiol Radiat Ther 2017;5:1072. [5] Pettersen JA, Aviv RI, Black SE, Fox AJ, Lim A, Murray BJ. Global hemispheric CT hypoperfusion may differentiate headache with associated neurological deficits and lymphocytosis from acute stroke. Stroke 2008;39:492–3. [6] Yilmaz A, Kaleagasi H, Dogu O, Kara E, Ozge A. Abnormal MRI in a patient with ’headache with neurological deficits and CSF lymphocytosis (HaNDL)’. Cephalalgia 2010;30:615–9. [7] Quintas S, López Ruiz R, Trillo S, Gago-Veiga AB, Zapata-Wainberg G, Dotor García-Soto J, et al. Clinical, imaging and electroencephalographic characterization of three cases of HaNDL syndrome. Cephalalgia 2017. https://doi.org/10.1177/0333102417735846 [Epub ahead of print]. [8] Miller C, Goldberg MF. Susceptibility-weighted imaging and computed tomography perfusion abnormalities in diagnosis of classic migraine. Emerg Radiol 2012;19:565–9. [9] Zamora CA, Kontzialis M. Susceptibility-weighted imaging in Todd paralysis. Neurology 2015;85:297. [10] Chen CY, Chen CI, Tsai FY, Tsai PH, Chan WP. Prominent vessel sign on susceptibility-weighted imaging in acute stroke: prediction of infarct growth and clinical outcome. PLoS One 2015;10:e0131118. [11] Mittal S, Wu Z, Neelavalli J, Haacke EM. Susceptibility-weighted imaging: technical aspects and clinical applications, Part 2. AJNR Am J Neuroradiol 2009;30:232–52. [12] Gómez-Aranda F, Cañadillas F, Martí-Massó JF, Díez-Tejedor E, Serrano PJ, Leira R, et al. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases. Brain 1997;120:1105–13.
Please cite this article as: C. Rodríguez-López, N. Garzo Caldas, D. Uriarte Pérez de Urabayen et al., A new MR radiological sign in HaNDL syndrome. A case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2018.11.018
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
A new MR radiological sign in HaNDL syndrome. A case report Claudia Rodríguez-López a,⇑,1, Nicolás Garzo Caldas a,1, David Uriarte Pérez de Urabayen a, Mario Sánchez Tornero a, Amaya Hilario Barrio b, Rosana Saiz Díaz a, Jesús González de la Aleja a a b
Department of Neurology, 12 de Octubre University Hospital, Madrid, Spain Department of Neuroradiology, 12 de Octubre University Hospital, Madrid, Spain
a r t i c l e
i n f o
Article history: Received 18 July 2018 Accepted 5 November 2018 Available online xxxx Keywords: HaNDL syndrome Susceptibility weighed MR Prominence vessel sign Oxygen extraction fraction
a b s t r a c t Background: One of the classical diagnostic criteria of the syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL syndrome) is the normality of imaging studies except from some reversible alterations as leptomeningeal enhancement or focal hypoperfusion. Case: We present a 41 year-old man who abruptly started with a set of right parietal symptoms, meeting the diagnostic criteria for HaNDL syndrome. An electroencephalographic record showed a slowing of the right hemisphere. MR susceptibility weighted sequences demonstrated a reduced venous signal in the symptomatic hemisphere, unlike other transient disorders as migraine aura where an opposite pattern with prominence of the venous structures in the symptomatic hemisphere has been reported. This sign could reflect a decrease in metabolic demands or a fail in oxygen employment by the affected tissue. Conclusion: To our knowledge, this finding has not been described yet, and it may provide a new insight on the pathogenesis of HaNDL syndrome. Ó 2018 Elsevier Ltd. All rights reserved.
1. Introduction The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL syndrome) [1] remains as a disorder of unknown origin and a poorly understood pathogenesis. One of its classical diagnostic criteria is the normality of all the imaging studies; nevertheless, some reversible alterations have been recently described. We present a case of HaNDL syndrome with new and unique radiological findings. 2. Case report A 41 years-old man without vascular risk factors nor antecedents of migraine presented with sudden, intense headache, right hemiparesis and motor aphasia which resolved spontaneously after 80 min. He was previously diagnosed of transient ischemic attack in another hospital and was referred to a stroke prevention clinic. Three days later he developed an identical set of symptoms lasting 60 min and after a period of three hours free from focality, he started with inattention, left central facial paresis, left hemianopia and left alien limb. This situation was associated again with striking headache as well as fever (38 °C). During the symptomatic ⇑ Corresponding author at: Department of Neurology, 12 de Octubre University Hospital, Avenida de Córdoba S/N, 28041 Madrid, Spain. E-mail address: [email protected] (C. Rodríguez-López). 1 Both authors contributed equally.
period the following studies were performed: firstly, a basal CT showed no alterations and an angioCT excluded intracranial occlusions. MR discarded restricted diffusion and revealed leptomeningeal enhancement in FLAIR sequences with contrast (Fig. 1A) as well as decreased prominence of the venous vasculature within the right hemisphere (Fig. 1B) in susceptibility weighted imaging (SWI). Finally, a lumbar puncture showed sterile lymphocytic pleocytosis (40 cells, 90% mononuclears) with hyperproteinorrhaquia (1.86 g/L), and the electroencephalogram demonstrated an asymmetrical diffuse slowing, more pronounced in the right hemisphere (Fig. 2A). Laboratory workup was normal. Extensive serum and CSF microbiological tests were negative. He was diagnosed of HaNDL syndrome according to the International Classification of Headache Disorders, 3rd edition (ICHD-3) [2]. His symptoms completely solved after 48 h, parallel to electroencephalogram normalization (Fig. 2B), without any specific treatment but analgesics. Eight days later he experimented once again right hemiparesis and motor aphasia in the set of intense headache with autolimitation in 60 min, and he was discharged with corticosteroid treatment in descending regimen. He has remained asymptomatic during a year follow-up.
3. Discussion Neuroimaging in HaNDL syndrome is usually normal; however some nonspecific abnormalities have been reported, such as mild
https://doi.org/10.1016/j.jocn.2018.11.018 0967-5868/Ó 2018 Elsevier Ltd. All rights reserved.
Please cite this article as: C. Rodríguez-López, N. Garzo Caldas, D. Uriarte Pérez de Urabayen et al., A new MR radiological sign in HaNDL syndrome. A case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2018.11.018
2
Case report / Journal of Clinical Neuroscience xxx (xxxx) xxx
Fig. 1. Axial MR images during the symptomatic right parietal syndrome. (A) FLAIR sequence with contrast revealed leptomeningeal enhancement at different levels. (B) SWI showed a normal prominence of venous structures within the left hemisphere but a reduced prominence of the venous vasculature within the right one at different levels.
Fig. 2. Electroencephalographic records of the patient. (A) During the symptomatic right parietal syndrome an asymmetrical persistent bilateral slowing was identified. A poorly organized background can be appreciated, as well as irregular and diffuse theta activity intermixed with delta activity, being more pronounced on the right hemisphere. (B) One day after remaining asymptomatic, electroencephalographic normalization was demonstrated.
Please cite this article as: C. Rodríguez-López, N. Garzo Caldas, D. Uriarte Pérez de Urabayen et al., A new MR radiological sign in HaNDL syndrome. A case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2018.11.018
Case report / Journal of Clinical Neuroscience xxx (xxxx) xxx
leptomeningeal enhancement [3,4] or focal regions of hypoperfusion that correlated with the neurological deficits [5–7]. In our case, no perfusion techniques were performed. Nevertheless, a SWI was carried out in search of alterations, since prominence of the venous vasculature (prominence vessel sign, PVS) within the symptomatic brain region has been reported in other entities, such as migraine aura [8] or Todd palsy [9]. The PVS in the draining veins within areas of impaired perfusion has also been described in ischemic stroke [10], and it has been interpreted as the uncoupling between oxygen supply and demand in hypoperfused tissue, which would cause a relative increase of deoxyhemoglobin levels and a decrease of oxyhemoglobin in the tissue capillaries and the draining veins [11]. Surprisingly, our patient presented an opposite pattern, with reduced venous signal in the symptomatic hemisphere where we also observed a focal electroencephalographic slowing. This reduced venous signal, possibly caused by the decreased oxygen extraction fraction, could indicate a decrease in metabolic demands or a fail in oxygen employment by the affected tissue. Thus, the mechanism responsible for the deficits in HaNDL syndrome could be different to that known in migraine aura or postictal neurological deficits. In any case, given the unknown etiology and pathophysiology of HaNDL syndrome, this finding raises a number of interesting questions. Finally, we would like to remark the atypical nature of the symptoms of this patient, since the characteristic manifestations of HaNDL syndrome are hemimotor, hemisensory and aphasic deficits [12], but an isolated parietal syndrome has not been described to date in this entity to our knowledge. 4. Conclusion If confirmed, the reduced venous signal within the symptomatic region seen in SWI could represent an useful clinical tool in the diagnosis of HaNDL syndrome, since it has not been described in any other else to our knowledge, and it could suggest a different pathophysiology underlying HaNDL syndrome.
3
Acknowledgements This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. The authors declare that there is no conflict of interest. References [1] Berg MJ, Williams LS. The transient syndrome of headache with neurologic deficits and CSF lymphocytosis. Neurology 1995;45:1648–54. [2] Headache Classification Committee of the International Headache Society (IHS). The International classification of headache disorders, 3rd edition. Cephalalgia 2013;33:629–808. [3] Emond H, Schnorf H, Poloni C, Vulliemoz S, Lalive PH. Syndrome of transient headache and neurological deficits with CSF lymphocytosis (HaNDL) associated with recent human herpesvirus-6 infection. Cephalalgia 2009;29:487–91. [4] Patel N, Gupta V, Barrett K, Vibhute P. A syndrome of headache and neurological deficits with cerebrospinal fluid (CSF) lymphocytic pleocytosis (Handl) with diffuse vasogenic leakage of gadolinium MRI contrast into the extra-axial CSF cisterns. J Radiol Radiat Ther 2017;5:1072. [5] Pettersen JA, Aviv RI, Black SE, Fox AJ, Lim A, Murray BJ. Global hemispheric CT hypoperfusion may differentiate headache with associated neurological deficits and lymphocytosis from acute stroke. Stroke 2008;39:492–3. [6] Yilmaz A, Kaleagasi H, Dogu O, Kara E, Ozge A. Abnormal MRI in a patient with ’headache with neurological deficits and CSF lymphocytosis (HaNDL)’. Cephalalgia 2010;30:615–9. [7] Quintas S, López Ruiz R, Trillo S, Gago-Veiga AB, Zapata-Wainberg G, Dotor García-Soto J, et al. Clinical, imaging and electroencephalographic characterization of three cases of HaNDL syndrome. Cephalalgia 2017. https://doi.org/10.1177/0333102417735846 [Epub ahead of print]. [8] Miller C, Goldberg MF. Susceptibility-weighted imaging and computed tomography perfusion abnormalities in diagnosis of classic migraine. Emerg Radiol 2012;19:565–9. [9] Zamora CA, Kontzialis M. Susceptibility-weighted imaging in Todd paralysis. Neurology 2015;85:297. [10] Chen CY, Chen CI, Tsai FY, Tsai PH, Chan WP. Prominent vessel sign on susceptibility-weighted imaging in acute stroke: prediction of infarct growth and clinical outcome. PLoS One 2015;10:e0131118. [11] Mittal S, Wu Z, Neelavalli J, Haacke EM. Susceptibility-weighted imaging: technical aspects and clinical applications, Part 2. AJNR Am J Neuroradiol 2009;30:232–52. [12] Gómez-Aranda F, Cañadillas F, Martí-Massó JF, Díez-Tejedor E, Serrano PJ, Leira R, et al. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases. Brain 1997;120:1105–13.
Please cite this article as: C. Rodríguez-López, N. Garzo Caldas, D. Uriarte Pérez de Urabayen et al., A new MR radiological sign in HaNDL syndrome. A case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2018.11.018