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The Syndrome of Headache with Neurologic Deficits and Cerebrospinal Fluid Lymphocytosis Mimicking Acute Ischemic Stroke
Case Report
The Syndrome of Headache with Neurologic Deficits and Cerebrospinal Fluid Lymphocytosis Mimicking Acute Ischemic Stroke Marcel J. H. Arie¨s, MD, Joost C. H. van Oostrom, MD, and Jacques de Keyser, MD, PhD
Headache with neurologic deficits and cerebrospinal fluid lymphocytosis is a benign condition with a transient ischemic attack—like presentation. We present a case of a 22-year-old man with episodes of right-sided weakness, global dysphasia, and right-sided homonymous hemianopsia associated with frequent vomiting. Systemic thrombolytic therapy was considered. A subsequent magnetic resonance image with diffusion-weighted images revealed normal findings. A diagnosis of headache with neurologic deficits and cerebrospinal fluid lymphocytosis was made based mainly on clinical symptoms and cerebrospinal fluid analysis. With the current pressure on ‘‘door-to-needle’’ time for thrombolysis, it is important to keep an open eye to stroke mimics and exclude them if possible. We, therefore, strongly advise considering noninvasive magnetic resonance diffusion-weighted imaging in patients with unusual presentations (e.g., the vomiting in our patient) before thrombolysis. Key Words: Stroke mimic—pseudomigraine—headache—magnetic resonance imaging—diffusion-weighted imaging. Ó 2008 by National Stroke Association
Headache with neurologic deficits and cerebrospinal fluid (CSF) lymphocytosis (HaNDL) is a rare condition that can present with strokelike manifestations before the diagnosis is suggested by the occurrence of migrainelike headache and CSF lymphocytosis. This can lead to therapeutic dilemmas with regard to thrombolysis.
From the Department of Neurology, University Medical Centre Groningen, University of Groningen, The Netherlands. Received November 30, 2007; revision received February 5, 2008; accepted February 13, 2008. Address correspondence to Marcel J. H. Arie¨s, MD, Department of Neurology, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9713 EZ Groningen, The Netherlands. E-mail: [email protected]. 1052-3057/$—see front matter Ó 2008 by National Stroke Association doi:10.1016/j.jstrokecerebrovasdis.2008.02.010
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Case Report A 22-year-old, right-handed, previously healthy man was admitted to the emergency department with sudden loss of speech and frequent vomiting. His friends said that he had used 3 U of alcohol that evening but no other drugs. He was not taking any medication. Examination revealed a puzzled, pale, vomiting man with normal vital signs, global dysphasia, right-sided homonymous hemianopsia, and paresis of the right arm. A toxicologic screening produced negative findings. A brain computed tomography scan revealed no abnormality and systemic thrombolytic therapy was considered. However, because of the vomiting and the patient’s age, we urgently performed brain magnetic resonance imaging, including diffusion-weighted imaging and magnetic resonance angiography. All imaging studies produced normal results and the thrombolysis protocol was stopped. The patient was admitted and antiemetics
Journal of Stroke and Cerebrovascular Diseases, Vol. 17, No. 4 (July-August), 2008: pp 246-247
HANDL MIMICKING STROKE
were started. Eight hours after the start of his neurologic symptoms, he experienced bilateral temporal headache with ongoing vomiting. CSF examination showed 49 mononuclear cells/mm3 with normal total protein and glucose levels. Ten hours after onset of the aphasia only a slight bilateral headache persisted. He was not known to have migraine attacks, but his mother experienced migraine without aura.
Discussion Because systemic thrombolysis carries a risk of hemorrhage it is important to differentiate between ischemic stroke and more benign causes of sudden neurologic deficits. HaNDL, also referred to as ‘‘pseudomigraine with temporary neurologic symptoms and lymphocytic pleocytosis’’ is a benign and self-limiting syndrome.1-3 The origin of HaNDL remains uncertain, but similarities between familial hemiplegic migraine and HaNDL suggest that it might be a calcium channelopathy.4 HaNDL should be suspected in young adults with recurrent episodes of moderate to severe bilateral or hemicranial headache accompanied by changing transient neurologic deficits, and complete resolution of the recurrent episodes within 2 months with absence of symptoms between the episodes. Exclusion of ischemic and infectious causes is essential for the diagnosis. CSF lymphocytic pleocytosis with negative causative results confirms the diagnosis.1,3 Distinguishing HaNDL from (transient) ischemic attacks, epileptic seizures, and meningoencephalitis is the first diagnostic step and makes elaborate neuroradiologic studies in the acute setting inevitable. Our patient presented with symptoms suggestive of acute ischemic stroke. With the current pressure on ‘‘door-to-needle’’ time for thrombolysis, it is important to keep an open eye to stroke mimics and exclude them if possible. Diffusion-weighted magnetic resonance imaging appears
247
highly accurate in acute ischemic stroke of less than 6 hours,5 but false-negative findings can occur with small ischemic lesions, especially in the brainstem.6 In our patient, the excessive vomiting was a red-flag symptom, difficult to explain on the basis of an acute partial anterior circulation ischemic stroke. Vomiting is uncommon in anterior circulation ischemic stroke (3.5%),7 and is then usually seen in extensive ischemia producing mass effect. Interestingly, in a recent study, vomiting was not identified as a clinical feature to distinguish between stroke and its mimics.8
References 1. Gomez-Aranda AF, Canadillas J, Marti-Masso´ JF, et al. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis: A report of 50 cases. Brain 1997;1120:1105-1113. 2. Kirchman Eriksen M, Thomsen LL, Olesen J. Implications of clinical subtypes of migraine with aura. Headache 2006; 46:286-297. 3. Landsberg MC. Headache with neurological deficits and CSF lymphocytosis: A transient ischemic attack mimic. J Stroke Cerebrovasc Dis 1999;8:42-44. 4. Ducros A, Denier C, Joutel A, et al. The clinical spectrum of familial hemiplegic migraine associated with mutations in a neuronal calcium channel. N Engl J Med 2001; 59:1016-1018. 5. Gonzalez RG, Schaefer PW, Buonanno FS, et al. Diffusionweighted MR imaging: Diagnostic accuracy in patients imaged within 6 hours of stroke symptom onset. Radiology 1999;210:155-162. 6. Oppenheim C, Stanescu R, Dormont D, et al. False negative diffusion-weighted MR findings in acute ischemic stroke. AJNR Am J Neuroradiol 2000;21:1434-1440. 7. Libman RB, Kwiatkowski TG, Hansen MD, et al. Differences between anterior and posterior circulation stroke in TOAST. Cerebrovasc Dis 2001;11:311-316. 8. Hand PJ, Kwan J, Lindley RI, et al. Distinguishing between stroke and mimic at the bedside: The brain attack study. Stroke 2006;37:769-775.
The Syndrome of Headache with Neurologic Deficits and Cerebrospinal Fluid Lymphocytosis Mimicking Acute Ischemic Stroke Marcel J. H. Arie¨s, MD, Joost C. H. van Oostrom, MD, and Jacques de Keyser, MD, PhD
Headache with neurologic deficits and cerebrospinal fluid lymphocytosis is a benign condition with a transient ischemic attack—like presentation. We present a case of a 22-year-old man with episodes of right-sided weakness, global dysphasia, and right-sided homonymous hemianopsia associated with frequent vomiting. Systemic thrombolytic therapy was considered. A subsequent magnetic resonance image with diffusion-weighted images revealed normal findings. A diagnosis of headache with neurologic deficits and cerebrospinal fluid lymphocytosis was made based mainly on clinical symptoms and cerebrospinal fluid analysis. With the current pressure on ‘‘door-to-needle’’ time for thrombolysis, it is important to keep an open eye to stroke mimics and exclude them if possible. We, therefore, strongly advise considering noninvasive magnetic resonance diffusion-weighted imaging in patients with unusual presentations (e.g., the vomiting in our patient) before thrombolysis. Key Words: Stroke mimic—pseudomigraine—headache—magnetic resonance imaging—diffusion-weighted imaging. Ó 2008 by National Stroke Association
Headache with neurologic deficits and cerebrospinal fluid (CSF) lymphocytosis (HaNDL) is a rare condition that can present with strokelike manifestations before the diagnosis is suggested by the occurrence of migrainelike headache and CSF lymphocytosis. This can lead to therapeutic dilemmas with regard to thrombolysis.
From the Department of Neurology, University Medical Centre Groningen, University of Groningen, The Netherlands. Received November 30, 2007; revision received February 5, 2008; accepted February 13, 2008. Address correspondence to Marcel J. H. Arie¨s, MD, Department of Neurology, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9713 EZ Groningen, The Netherlands. E-mail: [email protected]. 1052-3057/$—see front matter Ó 2008 by National Stroke Association doi:10.1016/j.jstrokecerebrovasdis.2008.02.010
246
Case Report A 22-year-old, right-handed, previously healthy man was admitted to the emergency department with sudden loss of speech and frequent vomiting. His friends said that he had used 3 U of alcohol that evening but no other drugs. He was not taking any medication. Examination revealed a puzzled, pale, vomiting man with normal vital signs, global dysphasia, right-sided homonymous hemianopsia, and paresis of the right arm. A toxicologic screening produced negative findings. A brain computed tomography scan revealed no abnormality and systemic thrombolytic therapy was considered. However, because of the vomiting and the patient’s age, we urgently performed brain magnetic resonance imaging, including diffusion-weighted imaging and magnetic resonance angiography. All imaging studies produced normal results and the thrombolysis protocol was stopped. The patient was admitted and antiemetics
Journal of Stroke and Cerebrovascular Diseases, Vol. 17, No. 4 (July-August), 2008: pp 246-247
HANDL MIMICKING STROKE
were started. Eight hours after the start of his neurologic symptoms, he experienced bilateral temporal headache with ongoing vomiting. CSF examination showed 49 mononuclear cells/mm3 with normal total protein and glucose levels. Ten hours after onset of the aphasia only a slight bilateral headache persisted. He was not known to have migraine attacks, but his mother experienced migraine without aura.
Discussion Because systemic thrombolysis carries a risk of hemorrhage it is important to differentiate between ischemic stroke and more benign causes of sudden neurologic deficits. HaNDL, also referred to as ‘‘pseudomigraine with temporary neurologic symptoms and lymphocytic pleocytosis’’ is a benign and self-limiting syndrome.1-3 The origin of HaNDL remains uncertain, but similarities between familial hemiplegic migraine and HaNDL suggest that it might be a calcium channelopathy.4 HaNDL should be suspected in young adults with recurrent episodes of moderate to severe bilateral or hemicranial headache accompanied by changing transient neurologic deficits, and complete resolution of the recurrent episodes within 2 months with absence of symptoms between the episodes. Exclusion of ischemic and infectious causes is essential for the diagnosis. CSF lymphocytic pleocytosis with negative causative results confirms the diagnosis.1,3 Distinguishing HaNDL from (transient) ischemic attacks, epileptic seizures, and meningoencephalitis is the first diagnostic step and makes elaborate neuroradiologic studies in the acute setting inevitable. Our patient presented with symptoms suggestive of acute ischemic stroke. With the current pressure on ‘‘door-to-needle’’ time for thrombolysis, it is important to keep an open eye to stroke mimics and exclude them if possible. Diffusion-weighted magnetic resonance imaging appears
247
highly accurate in acute ischemic stroke of less than 6 hours,5 but false-negative findings can occur with small ischemic lesions, especially in the brainstem.6 In our patient, the excessive vomiting was a red-flag symptom, difficult to explain on the basis of an acute partial anterior circulation ischemic stroke. Vomiting is uncommon in anterior circulation ischemic stroke (3.5%),7 and is then usually seen in extensive ischemia producing mass effect. Interestingly, in a recent study, vomiting was not identified as a clinical feature to distinguish between stroke and its mimics.8
References 1. Gomez-Aranda AF, Canadillas J, Marti-Masso´ JF, et al. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis: A report of 50 cases. Brain 1997;1120:1105-1113. 2. Kirchman Eriksen M, Thomsen LL, Olesen J. Implications of clinical subtypes of migraine with aura. Headache 2006; 46:286-297. 3. Landsberg MC. Headache with neurological deficits and CSF lymphocytosis: A transient ischemic attack mimic. J Stroke Cerebrovasc Dis 1999;8:42-44. 4. Ducros A, Denier C, Joutel A, et al. The clinical spectrum of familial hemiplegic migraine associated with mutations in a neuronal calcium channel. N Engl J Med 2001; 59:1016-1018. 5. Gonzalez RG, Schaefer PW, Buonanno FS, et al. Diffusionweighted MR imaging: Diagnostic accuracy in patients imaged within 6 hours of stroke symptom onset. Radiology 1999;210:155-162. 6. Oppenheim C, Stanescu R, Dormont D, et al. False negative diffusion-weighted MR findings in acute ischemic stroke. AJNR Am J Neuroradiol 2000;21:1434-1440. 7. Libman RB, Kwiatkowski TG, Hansen MD, et al. Differences between anterior and posterior circulation stroke in TOAST. Cerebrovasc Dis 2001;11:311-316. 8. Hand PJ, Kwan J, Lindley RI, et al. Distinguishing between stroke and mimic at the bedside: The brain attack study. Stroke 2006;37:769-775.