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Extensive meningeal enhancement in acute central nervous system Lyme: Case series and review of literature
25
Case Reports / Journal of Clinical Neuroscience 64 (2019) 25–27
Extensive meningeal enhancement in acute central nervous system Lyme: Case series and review of literature Mahbobeh Niksefat a, Bassam Albashiti a,b, Devin Burke b, Pouria Moshayedi b,⇑, Riddhi Patira b, Laurie Knepper b a b
Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, United States Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA, United States
a r t i c l e
i n f o
Article history: Received 20 February 2019 Accepted 25 March 2019
Keywords: CNS Lyme Magnetic resonance imaging Meningeal enhancement
a b s t r a c t Introduction. Clinical presentation of the central nervous system Lyme disease is nonspecific and therefore brain imaging and disease-specific serological testing is generally pursued to assist with diagnosis. Brain imaging findings are, however, rare and often unspecific. Case description. In the current report, we are presenting a rare magnetic resonance imaging (MRI) finding of extensive meningeal enhancement in two patients with acute Lyme disease. Discussion. We discussed clinical implications and reviewed the relevant literature.
1. Introduction Lyme disease, which is caused by the tick-borne spirochete Borrelia burgdorferi, is a multi-system infection and involves nervous system in 10–15% of cases [1]. It can cause cranial neuropathies, meningitis, encephalitis, increased intracranial pressure [2], and in rare cases facial pain syndromes [3]. Except cranial neuropathies, the clinical presentation of the nervous system Lyme is nonspecific. In these cases, brain imaging is generally performed to assist with diagnosis. However, magnetic resonance imaging (MRI) findings in Lyme are also nonspecific, and commonly include areas of parenchymal T2 signal changes and cranial nerve enhancement. In the current report we are presenting two cases with a rare MRI finding of extensive meningeal enhancement in patients with acute Lyme disease.
2. Case description 2.1. Case No 1 Patient was a 71 year-old man with history of two prior posterior cerebral artery territory infarcts, atrial fibrillation and prior alcohol abuse presented with 10 days of episodic dysarthria and right hand numbness lasting up to 1 h. The history was notable for 30 lb weight loss in the preceding 4 months, but negative for fevers, chills, skin rash, abdominal pain, nausea or diarrhea. His exam was nonremarkable except for mild confusion about time and place. Electroencephalography showed mild left posterior slowing attributed to the chronic left occipital infarction. Brain MRI with contrast was notable for lack of CSF suppression in T2 fluidattenuated inversion recovery (FLAIR) sequences (arrows in Fig. 1A), as well as leptomeningeal and pachymeningeal enhancement (arrowheads in Fig. 1B, C). Given the MRI finding, CSF analysis was performed and showed 45 WBCs (91% lymphocytes), 4 RBCs, protein 114 mg/dl and glucose 45 mg/dl (serum glucose 98 mg/dl). The other CSF parameters, including CSF opening pressure, cultures, viral PCRs, cryptococcal antigen, cytology, angiotenAbbreviations: CNS, central nervous system; CSF, cerebrospinal fluid; FLAIR, fluid-attenuated inversion recovery; MRI, magnetic resonance imaging. ⇑ Corresponding author. E-mail address: [email protected] (P. Moshayedi).
Ó 2019 Elsevier Ltd. All rights reserved.
sin converting enzyme titers as well as Borrelia Burgdorferi culture and PCR were negative. Serum tests for HIV, sarcoidosis, prostate specific antigen and protein electrophoresis did not yield any positive results. Chest CT images did not show any signs of infection or malignancy. Lyme serology was tested and revealed a positive titer of 3.87 index value (definite Lyme >1.10). Western blot showed 2 out of 3 positive IgM bands and 10 out of 10 positive IgG bands. Lyme serologic testing of CSF was not performed due to insufficient sample. He was discharged with oral doxycycline. 2.2. Case No 2 A 51 year-old man with history of cluster headaches initially presented to our facility with 10 days of persistent moderate daily headaches between episodic bouts of cluster headaches. Given lack of infectious signs or symptoms, and few migrainous properties to his headaches he was treated with dihydroergotamine injections and discharged after improvement in headaches. Improvement was, however, short-lived and headache returned after 24 h. He therefore sought medical attention, but brain and cervical spine MRI were unremarkable. CSF studies 6 weeks following onset of headaches revealed marked abnormalities with CSF pressure of 42 cmH2O, 180 WBC (100% lymphocytes), 5 RBC, protein 293 mg/dl and glucose 39 mg/dl (serum glucose 66 mg/dl). The serum Lyme screening test returned positive with over 12.0 index value (definite Lyme >1.10) and later Western blot confirmed presence of 3 out of 3 IgM bands and 9 out of 10 IgG. Lyme IgM and IgG antibodies were detected in the CSF (no titration was performed). Serum tests for HIV, syphilis, sarcoidosis, as well as CSF culture, cryptococcal antigen and viral PCR results were all negative. A repeat brain MRI with contrast revealed pachymeningeal enhancement (arrowheads in Fig. 1E and F), as well as contrast enhancement of bilateral 3rd and 5th cranial nerves (arrows in Fig. 1F). Patient was discharged to complete intravenous ceftriaxone injections. A follow up visit 2 weeks after commencement of treatment was notable for mild clinical improvement. 3. Discussion and conclusion Nervous system Lyme occurs in about 15% of patients with early manifestations of Lyme [4], and they often present with headaches, symptoms of meningitis (headache), cranial neuropathies (most
26
Case Reports / Journal of Clinical Neuroscience 64 (2019) 25–27
Fig. 1. Axial (A, B, D and E) and coronal (C and F) brain magnetic resonance images (MRI) in two patients with positive Lyme serology results. (A-C) shows brain MRI in the case No 1 with axial T2 fluid-attenuated inversion recovery (FLAIR) sequence with diffuse lack of CSF suppression (arrows in A) and contrast enhancement of leptomeningeal and pachymeningeal contrast enhancement (arrowheads in B and C). Brain MRI of Case No 2 are shown in D, E and F. Axial T2 FLAIR images in Case No 2 (D) are shown to compare with Case No 1. Contrasted MRI (E, D) in Case No 2 shows pachymeningeal enhancement (arrowheads) as well as enhancement of bilateral 5th cranial nerves (arrows).
commonly facial palsy) and radiculitis [5]. Besides these distinct presenting features of the nervous system Lyme, many present with nonspecific symptoms of headache, fatigue, cognitive dysfunction or language difficulties [6]. However, in order to attribute these nonspecific neurological symptoms to the central nervous system (CNS) involvement by Lyme, positive findings in the cerebrospinal fluid analysis or brain MRI is required [7]. Brain MRI is often the first test that is performed before cerebrospinal fluid analysis in patients presenting with nonspecific neurologic symptoms. MRI findings in neuroborreliosis are insensitive and nonspecific. A study showed that brain MRI is normal in a majority of patients with CNS Lyme [7] and in about 40% patients, there were nonspecific white matter T2 FLAIR areas of hyperintensities. Enhancement of cranial nerves, most specifically bilateral seventh cranial nerve, and nerve roots has also been reported and provides radiologic support for cranial neuropathies and radiculitis, respectively [8]. Myelitis has also been reported in late nervous system Lyme [9]. In another case series of 66 patients with CNS Lyme [10], MRI abnormalities were only seen in 11 cases (17%). Unspecific areas of T2 prolongation signal was relatively more frequent and seen in 7 cases (11%). A very mild meningeal enhancement was only seen in one case (1.5%). Meningeal enhancement is rare and mild, and restricted to the meninges at the base of skull [11–14] often in continuity with enhancement of cranial nerves. CNS Lyme can also cause cerebral vasculitis and stroke [15]. In both reported patients the insidious symptoms of headache and intermittent language and sensory deficits led to imaging findings of extensive leptomeningeal and pachymeningeal enhancement suggesting inflammation in pia and dura-arachnoid layers, respectively. CSF analysis confirmed a lymphocytedominant pleocytosis with a broad differential diagnosis of insidious meningeal infections. However, positive Lyme serology results in serum (cases 1 and 2) and CSF (case 2) confirmed the diagnosis of CNS Lyme.
At the conclusion, we recommend CNS Lyme to be considered as one differential diagnosis of meningeal enhancement, especially in endemic areas for Lyme. Declarations of interest None. Funding statement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. References [1] Koedel U, Pfister HW. Lyme neuroborreliosis. Curr Opin Infect Dis 2017;30:101–7. [2] Halperin JJ. Nervous system Lyme disease. Clin Lab Med 2015;35:779–95. [3] Ebner D, Smith K, DeSimone D, Sohail MR. Cranial neuropathy and severe pain due to early disseminated Borrelia burgdorferi infection. BMJ Case Rep 2018;2018. [4] Marques AR. Lyme neuroborreliosis. Continuum (Minneap Minn) 2015;21:1729–44. [5] Oschmann P, Dorndorf W, Hornig C, Schafer C, Wellensiek HJ, Pflughaupt KW. Stages and syndromes of neuroborreliosis. J Neurol 1998;245:262–72. [6] Halperin JJ, Pass HL, Anand AK, Luft BJ, Volkman DJ, Dattwyler RJ. Nervous system abnormalities in Lyme disease. Ann N Y Acad Sci 1988;539:24–34. [7] Halperin JJ, Luft BJ, Anand AK, Roque CT, Alvarez O, Volkman DJ, et al. Lyme neuroborreliosis: central nervous system manifestations. Neurology 1989;39:753–9. [8] Hildenbrand P, Craven DE, Jones R, Nemeskal P. Lyme neuroborreliosis: manifestations of a rapidly emerging zoonosis. AJNR Am J Neuroradiol 2009;30:1079–87. [9] Beuchat I, Dunet V, Meylan P, Du Pasquier R. Late Lyme neuroborreliosis with chronic encephalomyelitis. Neurology 2018;91:627–8. [10] Agarwal R, Sze G. Neuro-lyme disease: MR imaging findings. Radiology 2009;253:167–73. [11] Demaerel P, Wilms G, Van Lierde S, Delanote J, Baert AL. Lyme disease in childhood presenting as primary leptomeningeal enhancement without parenchymal findings on MR. AJNR Am J Neuroradiol 1994;15:302–4.
Case Reports / Journal of Clinical Neuroscience 64 (2019) 27–29 [12] Fischer S, Weber J, Senn-Schonenberger I, Cerny T, Hundsberger T. Neuroborreliosis mimicking leptomeningeal carcinomatosis in a patient with breast cancer: a case report. J Investig Med High Impact Case Rep 2014;2. 2324709614529417. [13] Nelson JA, Wolf MD, Yuh WT, Peeples ME. Cranial nerve involvement with Lyme borreliosis demonstrated by magnetic resonance imaging. Neurology 1992;42:671–3.
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[14] Pachner AR, Delaney E, O’Neill T, Major E. Inoculation of nonhuman primates with the N40 strain of Borrelia burgdorferi leads to a model of Lyme neuroborreliosis faithful to the human disease. Neurology 1995;45:165–72. [15] Topakian R, Stieglbauer K, Nussbaumer K, Aichner FT. Cerebral vasculitis and stroke in Lyme neuroborreliosis. Two case reports and review of current knowledge. Cerebrovasc Dis 2008;26:455–61.
https://doi.org/10.1016/j.jocn.2019.03.048
Cerebral embolism and carotid-hyoid impingement syndrome Lay Kun Kho a,b,⇑, Timothy R Bates b, Andrew Thompson c, Ferry Dharsono c, David Prentice a a b c
Royal Perth Hospital, Western Australia, Australia Midland St John Of God Hospital, Western Australia, Australia Neurological Intervention & Imaging Service of WA (NIISwa), Western Australia, Australia
a r t i c l e
i n f o
Article history: Received 1 August 2018 Accepted 22 March 2019
Keywords: Embolic stroke Hyoid bone Carotid-hyoid syndrome
a b s t r a c t Embolic stroke of undetermined source (ESUS) is not uncommon in young patients. Here, we describe two cases of stroke from an unusual aetiology; cerebral embolization from carotid artery injury presumed secondary to hyoid bone impingement. Both patients demonstrated angiographic evidence of hyoid bone impingement. Following resection of the greater cornu of the hyoid bone, neither patient had further strokes. Ó 2019 Published by Elsevier Ltd.
1. Introduction Embolic stroke of undetermined source (ESUS) is not uncommon in young patients. Extensive investigations may not determine the cause of stroke [1]. This uncertainty is distressing for patients and frustrating for clinicians. We describe 2 cases with an unusual cause of stroke; that being cerebral emboli from carotid artery injury secondary to presumed impingement from the hyoid bone. 2. Case reports 2.1. Case 1 29-year old woman presented with intermittent episodes of expressive dysphasia for six weeks. Two weeks prior, she had uncomplicated drainage of left upper incisor dental abscess. On examination, she was afebrile, normotensive with normal neurological examination. MRI showed multiple infarcts in left middle and anterior cerebral artery territories with her left internal carotid artery (ICA) in normal position i.e. anterolateral to external carotid artery (ECA). Subsequent CT angiogram (CTA) showed minor irregularities of the proximal left ICA but the ICA was now postero-medial to ECA and behind the greater cornu of the hyoid (Fig. 1). Extensive workup (Transthoracic Echocardiogram, Transoesophageal Echocardiogram, Holter, thrombophilia, lumbar puncture and autoimmune screens) was normal. She was discharged on Aspirin 100 mg/day. Five months later she presented with right arm weakness and dysarthria lasting 30 min. She also noticed four episodes of right ⇑ Corresponding author at: Department of Neurology, Level 7, Royal Perth Hospital, Wellington St, Perth, WA 6000, Australia. E-mail addresses: [email protected] (L.K. Kho), [email protected] (T.R Bates), [email protected] (A. Thompson), Ferry.Dharsono@ health.wa.gov.au (F. Dharsono), [email protected] (D. Prentice).
leg weakness lasting several minutes over the preceding two months. This time, her MRI showed new areas of infarction on DWI sequences in left MCA territory. Further history revealed that prior to the first stroke, the patient had developed a nervous habit of ‘‘clicking her neck” by rapid rotation from left to right, up to ten times a day for three months. The second presentation to hospital occurred after falling asleep on the couch for eight hours lying on her left side in an awkward position. She proceeded to a cerebral angiogram with head repositioning. On extreme left lateral head tilt, there was medial subluxation of the left ICA to behind the greater cornu of the hyoid bone. She was commenced on Clopidogrel 75 mg/day. An otolaryngologist was consulted and resection of the left greater cornu of the hyoid was performed. At operation a dense fibrous band connected the tip of the hyoid to superior cornu of thyroid cartilage. Histopathology was normal. She has had no recurrent events.
2.2. Case 2 A 34-year old woman presented with sudden onset dysarthria and left facial droop. In the preceding fortnight, she reported a constant right earache that previously had been intermittent for two years. This pain radiated to the mid-sternomastoid area. It was unchanged by deglutition or phonation, but was worse in cold weather or pressure over the hyoid area. There was no history of neck injury or manipulation, with no known conventional vascular risk factors. Her only medication was the oral contraceptive pill. Initial MRI revealed DWI hyperintensities consistent with multiple infarcts in the right frontal lobe. Contrast-enhanced CTA showed a shelf-like protuberance from the medial wall of the right carotid artery, just above the carotid bifurcation (Fig. 2). Extensive workup for alternative causes of stroke was unremarkable. Patient was referred for vessel-wall imaging with head repositioning due to the neck pain and close proximity of the hyoid bone. This showed the right ICA to be hypermobile. In the neutral
Case Reports / Journal of Clinical Neuroscience 64 (2019) 25–27
Extensive meningeal enhancement in acute central nervous system Lyme: Case series and review of literature Mahbobeh Niksefat a, Bassam Albashiti a,b, Devin Burke b, Pouria Moshayedi b,⇑, Riddhi Patira b, Laurie Knepper b a b
Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, United States Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA, United States
a r t i c l e
i n f o
Article history: Received 20 February 2019 Accepted 25 March 2019
Keywords: CNS Lyme Magnetic resonance imaging Meningeal enhancement
a b s t r a c t Introduction. Clinical presentation of the central nervous system Lyme disease is nonspecific and therefore brain imaging and disease-specific serological testing is generally pursued to assist with diagnosis. Brain imaging findings are, however, rare and often unspecific. Case description. In the current report, we are presenting a rare magnetic resonance imaging (MRI) finding of extensive meningeal enhancement in two patients with acute Lyme disease. Discussion. We discussed clinical implications and reviewed the relevant literature.
1. Introduction Lyme disease, which is caused by the tick-borne spirochete Borrelia burgdorferi, is a multi-system infection and involves nervous system in 10–15% of cases [1]. It can cause cranial neuropathies, meningitis, encephalitis, increased intracranial pressure [2], and in rare cases facial pain syndromes [3]. Except cranial neuropathies, the clinical presentation of the nervous system Lyme is nonspecific. In these cases, brain imaging is generally performed to assist with diagnosis. However, magnetic resonance imaging (MRI) findings in Lyme are also nonspecific, and commonly include areas of parenchymal T2 signal changes and cranial nerve enhancement. In the current report we are presenting two cases with a rare MRI finding of extensive meningeal enhancement in patients with acute Lyme disease.
2. Case description 2.1. Case No 1 Patient was a 71 year-old man with history of two prior posterior cerebral artery territory infarcts, atrial fibrillation and prior alcohol abuse presented with 10 days of episodic dysarthria and right hand numbness lasting up to 1 h. The history was notable for 30 lb weight loss in the preceding 4 months, but negative for fevers, chills, skin rash, abdominal pain, nausea or diarrhea. His exam was nonremarkable except for mild confusion about time and place. Electroencephalography showed mild left posterior slowing attributed to the chronic left occipital infarction. Brain MRI with contrast was notable for lack of CSF suppression in T2 fluidattenuated inversion recovery (FLAIR) sequences (arrows in Fig. 1A), as well as leptomeningeal and pachymeningeal enhancement (arrowheads in Fig. 1B, C). Given the MRI finding, CSF analysis was performed and showed 45 WBCs (91% lymphocytes), 4 RBCs, protein 114 mg/dl and glucose 45 mg/dl (serum glucose 98 mg/dl). The other CSF parameters, including CSF opening pressure, cultures, viral PCRs, cryptococcal antigen, cytology, angiotenAbbreviations: CNS, central nervous system; CSF, cerebrospinal fluid; FLAIR, fluid-attenuated inversion recovery; MRI, magnetic resonance imaging. ⇑ Corresponding author. E-mail address: [email protected] (P. Moshayedi).
Ó 2019 Elsevier Ltd. All rights reserved.
sin converting enzyme titers as well as Borrelia Burgdorferi culture and PCR were negative. Serum tests for HIV, sarcoidosis, prostate specific antigen and protein electrophoresis did not yield any positive results. Chest CT images did not show any signs of infection or malignancy. Lyme serology was tested and revealed a positive titer of 3.87 index value (definite Lyme >1.10). Western blot showed 2 out of 3 positive IgM bands and 10 out of 10 positive IgG bands. Lyme serologic testing of CSF was not performed due to insufficient sample. He was discharged with oral doxycycline. 2.2. Case No 2 A 51 year-old man with history of cluster headaches initially presented to our facility with 10 days of persistent moderate daily headaches between episodic bouts of cluster headaches. Given lack of infectious signs or symptoms, and few migrainous properties to his headaches he was treated with dihydroergotamine injections and discharged after improvement in headaches. Improvement was, however, short-lived and headache returned after 24 h. He therefore sought medical attention, but brain and cervical spine MRI were unremarkable. CSF studies 6 weeks following onset of headaches revealed marked abnormalities with CSF pressure of 42 cmH2O, 180 WBC (100% lymphocytes), 5 RBC, protein 293 mg/dl and glucose 39 mg/dl (serum glucose 66 mg/dl). The serum Lyme screening test returned positive with over 12.0 index value (definite Lyme >1.10) and later Western blot confirmed presence of 3 out of 3 IgM bands and 9 out of 10 IgG. Lyme IgM and IgG antibodies were detected in the CSF (no titration was performed). Serum tests for HIV, syphilis, sarcoidosis, as well as CSF culture, cryptococcal antigen and viral PCR results were all negative. A repeat brain MRI with contrast revealed pachymeningeal enhancement (arrowheads in Fig. 1E and F), as well as contrast enhancement of bilateral 3rd and 5th cranial nerves (arrows in Fig. 1F). Patient was discharged to complete intravenous ceftriaxone injections. A follow up visit 2 weeks after commencement of treatment was notable for mild clinical improvement. 3. Discussion and conclusion Nervous system Lyme occurs in about 15% of patients with early manifestations of Lyme [4], and they often present with headaches, symptoms of meningitis (headache), cranial neuropathies (most
26
Case Reports / Journal of Clinical Neuroscience 64 (2019) 25–27
Fig. 1. Axial (A, B, D and E) and coronal (C and F) brain magnetic resonance images (MRI) in two patients with positive Lyme serology results. (A-C) shows brain MRI in the case No 1 with axial T2 fluid-attenuated inversion recovery (FLAIR) sequence with diffuse lack of CSF suppression (arrows in A) and contrast enhancement of leptomeningeal and pachymeningeal contrast enhancement (arrowheads in B and C). Brain MRI of Case No 2 are shown in D, E and F. Axial T2 FLAIR images in Case No 2 (D) are shown to compare with Case No 1. Contrasted MRI (E, D) in Case No 2 shows pachymeningeal enhancement (arrowheads) as well as enhancement of bilateral 5th cranial nerves (arrows).
commonly facial palsy) and radiculitis [5]. Besides these distinct presenting features of the nervous system Lyme, many present with nonspecific symptoms of headache, fatigue, cognitive dysfunction or language difficulties [6]. However, in order to attribute these nonspecific neurological symptoms to the central nervous system (CNS) involvement by Lyme, positive findings in the cerebrospinal fluid analysis or brain MRI is required [7]. Brain MRI is often the first test that is performed before cerebrospinal fluid analysis in patients presenting with nonspecific neurologic symptoms. MRI findings in neuroborreliosis are insensitive and nonspecific. A study showed that brain MRI is normal in a majority of patients with CNS Lyme [7] and in about 40% patients, there were nonspecific white matter T2 FLAIR areas of hyperintensities. Enhancement of cranial nerves, most specifically bilateral seventh cranial nerve, and nerve roots has also been reported and provides radiologic support for cranial neuropathies and radiculitis, respectively [8]. Myelitis has also been reported in late nervous system Lyme [9]. In another case series of 66 patients with CNS Lyme [10], MRI abnormalities were only seen in 11 cases (17%). Unspecific areas of T2 prolongation signal was relatively more frequent and seen in 7 cases (11%). A very mild meningeal enhancement was only seen in one case (1.5%). Meningeal enhancement is rare and mild, and restricted to the meninges at the base of skull [11–14] often in continuity with enhancement of cranial nerves. CNS Lyme can also cause cerebral vasculitis and stroke [15]. In both reported patients the insidious symptoms of headache and intermittent language and sensory deficits led to imaging findings of extensive leptomeningeal and pachymeningeal enhancement suggesting inflammation in pia and dura-arachnoid layers, respectively. CSF analysis confirmed a lymphocytedominant pleocytosis with a broad differential diagnosis of insidious meningeal infections. However, positive Lyme serology results in serum (cases 1 and 2) and CSF (case 2) confirmed the diagnosis of CNS Lyme.
At the conclusion, we recommend CNS Lyme to be considered as one differential diagnosis of meningeal enhancement, especially in endemic areas for Lyme. Declarations of interest None. Funding statement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. References [1] Koedel U, Pfister HW. Lyme neuroborreliosis. Curr Opin Infect Dis 2017;30:101–7. [2] Halperin JJ. Nervous system Lyme disease. Clin Lab Med 2015;35:779–95. [3] Ebner D, Smith K, DeSimone D, Sohail MR. Cranial neuropathy and severe pain due to early disseminated Borrelia burgdorferi infection. BMJ Case Rep 2018;2018. [4] Marques AR. Lyme neuroborreliosis. Continuum (Minneap Minn) 2015;21:1729–44. [5] Oschmann P, Dorndorf W, Hornig C, Schafer C, Wellensiek HJ, Pflughaupt KW. Stages and syndromes of neuroborreliosis. J Neurol 1998;245:262–72. [6] Halperin JJ, Pass HL, Anand AK, Luft BJ, Volkman DJ, Dattwyler RJ. Nervous system abnormalities in Lyme disease. Ann N Y Acad Sci 1988;539:24–34. [7] Halperin JJ, Luft BJ, Anand AK, Roque CT, Alvarez O, Volkman DJ, et al. Lyme neuroborreliosis: central nervous system manifestations. Neurology 1989;39:753–9. [8] Hildenbrand P, Craven DE, Jones R, Nemeskal P. Lyme neuroborreliosis: manifestations of a rapidly emerging zoonosis. AJNR Am J Neuroradiol 2009;30:1079–87. [9] Beuchat I, Dunet V, Meylan P, Du Pasquier R. Late Lyme neuroborreliosis with chronic encephalomyelitis. Neurology 2018;91:627–8. [10] Agarwal R, Sze G. Neuro-lyme disease: MR imaging findings. Radiology 2009;253:167–73. [11] Demaerel P, Wilms G, Van Lierde S, Delanote J, Baert AL. Lyme disease in childhood presenting as primary leptomeningeal enhancement without parenchymal findings on MR. AJNR Am J Neuroradiol 1994;15:302–4.
Case Reports / Journal of Clinical Neuroscience 64 (2019) 27–29 [12] Fischer S, Weber J, Senn-Schonenberger I, Cerny T, Hundsberger T. Neuroborreliosis mimicking leptomeningeal carcinomatosis in a patient with breast cancer: a case report. J Investig Med High Impact Case Rep 2014;2. 2324709614529417. [13] Nelson JA, Wolf MD, Yuh WT, Peeples ME. Cranial nerve involvement with Lyme borreliosis demonstrated by magnetic resonance imaging. Neurology 1992;42:671–3.
27
[14] Pachner AR, Delaney E, O’Neill T, Major E. Inoculation of nonhuman primates with the N40 strain of Borrelia burgdorferi leads to a model of Lyme neuroborreliosis faithful to the human disease. Neurology 1995;45:165–72. [15] Topakian R, Stieglbauer K, Nussbaumer K, Aichner FT. Cerebral vasculitis and stroke in Lyme neuroborreliosis. Two case reports and review of current knowledge. Cerebrovasc Dis 2008;26:455–61.
https://doi.org/10.1016/j.jocn.2019.03.048
Cerebral embolism and carotid-hyoid impingement syndrome Lay Kun Kho a,b,⇑, Timothy R Bates b, Andrew Thompson c, Ferry Dharsono c, David Prentice a a b c
Royal Perth Hospital, Western Australia, Australia Midland St John Of God Hospital, Western Australia, Australia Neurological Intervention & Imaging Service of WA (NIISwa), Western Australia, Australia
a r t i c l e
i n f o
Article history: Received 1 August 2018 Accepted 22 March 2019
Keywords: Embolic stroke Hyoid bone Carotid-hyoid syndrome
a b s t r a c t Embolic stroke of undetermined source (ESUS) is not uncommon in young patients. Here, we describe two cases of stroke from an unusual aetiology; cerebral embolization from carotid artery injury presumed secondary to hyoid bone impingement. Both patients demonstrated angiographic evidence of hyoid bone impingement. Following resection of the greater cornu of the hyoid bone, neither patient had further strokes. Ó 2019 Published by Elsevier Ltd.
1. Introduction Embolic stroke of undetermined source (ESUS) is not uncommon in young patients. Extensive investigations may not determine the cause of stroke [1]. This uncertainty is distressing for patients and frustrating for clinicians. We describe 2 cases with an unusual cause of stroke; that being cerebral emboli from carotid artery injury secondary to presumed impingement from the hyoid bone. 2. Case reports 2.1. Case 1 29-year old woman presented with intermittent episodes of expressive dysphasia for six weeks. Two weeks prior, she had uncomplicated drainage of left upper incisor dental abscess. On examination, she was afebrile, normotensive with normal neurological examination. MRI showed multiple infarcts in left middle and anterior cerebral artery territories with her left internal carotid artery (ICA) in normal position i.e. anterolateral to external carotid artery (ECA). Subsequent CT angiogram (CTA) showed minor irregularities of the proximal left ICA but the ICA was now postero-medial to ECA and behind the greater cornu of the hyoid (Fig. 1). Extensive workup (Transthoracic Echocardiogram, Transoesophageal Echocardiogram, Holter, thrombophilia, lumbar puncture and autoimmune screens) was normal. She was discharged on Aspirin 100 mg/day. Five months later she presented with right arm weakness and dysarthria lasting 30 min. She also noticed four episodes of right ⇑ Corresponding author at: Department of Neurology, Level 7, Royal Perth Hospital, Wellington St, Perth, WA 6000, Australia. E-mail addresses: [email protected] (L.K. Kho), [email protected] (T.R Bates), [email protected] (A. Thompson), Ferry.Dharsono@ health.wa.gov.au (F. Dharsono), [email protected] (D. Prentice).
leg weakness lasting several minutes over the preceding two months. This time, her MRI showed new areas of infarction on DWI sequences in left MCA territory. Further history revealed that prior to the first stroke, the patient had developed a nervous habit of ‘‘clicking her neck” by rapid rotation from left to right, up to ten times a day for three months. The second presentation to hospital occurred after falling asleep on the couch for eight hours lying on her left side in an awkward position. She proceeded to a cerebral angiogram with head repositioning. On extreme left lateral head tilt, there was medial subluxation of the left ICA to behind the greater cornu of the hyoid bone. She was commenced on Clopidogrel 75 mg/day. An otolaryngologist was consulted and resection of the left greater cornu of the hyoid was performed. At operation a dense fibrous band connected the tip of the hyoid to superior cornu of thyroid cartilage. Histopathology was normal. She has had no recurrent events.
2.2. Case 2 A 34-year old woman presented with sudden onset dysarthria and left facial droop. In the preceding fortnight, she reported a constant right earache that previously had been intermittent for two years. This pain radiated to the mid-sternomastoid area. It was unchanged by deglutition or phonation, but was worse in cold weather or pressure over the hyoid area. There was no history of neck injury or manipulation, with no known conventional vascular risk factors. Her only medication was the oral contraceptive pill. Initial MRI revealed DWI hyperintensities consistent with multiple infarcts in the right frontal lobe. Contrast-enhanced CTA showed a shelf-like protuberance from the medial wall of the right carotid artery, just above the carotid bifurcation (Fig. 2). Extensive workup for alternative causes of stroke was unremarkable. Patient was referred for vessel-wall imaging with head repositioning due to the neck pain and close proximity of the hyoid bone. This showed the right ICA to be hypermobile. In the neutral