Glob Pediatr Health. 2015; 2: 2333794X15601840.
Published online 2015 Aug 20. doi: 10.1177/2333794X15601840
Neuroborreliosis-Associated Cerebral Vasculitis
An Uncommon Manifestation of Lyme Disease in a 9-Year-Old Boy
Nina Lenherr, MD,1 Kathi Walther, MD,1 Jacques Schneider, MD,1 Andreas Woerner, MD,1 and Melanie Hess, MD
University Children’s Hospital, Basel, Switzerland
A 9-year-old boy was referred to our pediatric department with symptoms of chronic fatigue, headache, intermittent nausea, and vomiting for 2 months. At admission a weight loss of 2 kg and intermittent subfebril temperature as well as an unspecific tremor of both hands was reported. He had a medical history of erythema migrans 2 years ago treated adequately with amoxicillin for 2 weeks. There was no history of trauma or stay in subtropical countries. Family history revealed a Waldenstrom’s macroglobulinemia of his father and paternal uncle. Clinically, only mild postural tremor of both hands and mild horizontal nystagmus with no other clinical symptoms was noticed.
A cerebral magnetic resonance imaging (MRI) angiography was performed, showing 2 small acute cerebellar infarctions in the posterior inferior cerebellar artery area and occlusion of the basilar artery, highly suspicious of an underlying vasculitis (Figure 1A and andB).B). Conventional cerebral angiography detected a subtotal occlusion of the basilar and bilateral vertebral arteries with retrograde blood flow to the posterior cerebral circulation (Figure 1C).
Extended immunological, hematological, and endocrinological laboratory evaluation revealed no abnormalities, among others normal blood count; negative C-reactive protein; normal erythrocyte sedimentation rate; normal renal, thyroid, and liver function; negative thrombophilic parameters; and negative immunological tests with no contribution for systemic lupus erythematosus, antiphospholipid syndrome, or other rheumatologic diseases (Table 1). Instead, Borrelia burgdorferi serology was positive for IgM and IgG, with confirmation of 4 positive bands in Western blot. A lumbar puncture was performed, showing lymphocytosis with increased protein and decreased glucose levels along with an intrathecal production of Borrelia burgdorferi IgM antibodies.
Therefore, neuroborreliosis was assumed and treatment was started with intravenous ceftriaxone 80 mg/kg/day for 3 weeks in addition to oral acetylsalicylic acid (ASS) 5 mg/kg/day and prednisolone 1 mg/kg/day. Clinical symptoms subsequently improved and neurological examination returned to normal soon after therapy was started.
A follow-up lumbar puncture 3 weeks later showed a reduction of pleocytosis and normalization of liquor glucose and protein. Two months later, MRI angiography showed the absence of cerebellar diffusion restrictions, but an unchanged presentation of the basilar and bilateral vertebral arteries occlusion. The prednisolone therapy was tapered over 3 weeks, whereas ASS therapy was continued. Three months later, a subsequent MRI angiography showed an unvaried occlusion of the basilar and bilateral vertebral arteries with no areas of new infarction (Figure 1D). Clinical and radiological follow-up 6 and 12 months after diagnosis showed a boy without any residual symptoms but an unchanged MRI angiography with ongoing vascular occlusion.
Lyme disease is a tick-borne infection that is caused by gram-negative spirochetes, Borrelia species, that induce 3 manifestations: the early and localized presentation as erythema migrans (the most frequent form in children and adults); several early and disseminated forms like neuroborreliosis, lymphozytoma, arthritis, carditis, and myositis, manifesting within weeks; and late disseminated forms like acrodermatitis chronica atrophicans and others, presenting months after infection.1 Neuroborreliosis is one of the most common infections during childhood and diagnosis can be challenging because of its variety of noncharacteristic symptoms like long-lasting headache, fatigue, and loss of appetite up to presentations with acute hemiparesis.
Lyme disease has been only rarely identified as cause of ischemic or hemorrhagic stroke due to vasculitis in adults2,3 and even more rarely in children.4-6 Lesions due to vascular changes resulting from neuroborreliosis seem to be widely distributed within the brain, showing perfusion deficits due to infarction of the middle cerebral artery, the anterior and posterior artery, or of the central white matter and basal ganglia region.
Differential diagnosis of cerebral vasculitis of childhood is large. Beside systemic lupus erythematosus with central nervous system (CNS) involvement, clinical findings of fatigue, headache, and focal motor deficits are also frequently reported in childhood primary angiitis of the CNS, an entity of vasculitis restricted to the CNS with either anterior and/or posterior cerebral circulation involved.7 Signs of systemic inflammation such as C-reactive protein, erythrocyte sedimentation rate, white blood cells, or serum IgG elevation may be completely absent. Other differential diagnoses include genetically defined systemic inflammatory diseases with predominant CNS manifestation,8 antibody-mediated brain inflammation,9 and metabolic diseases with inflammation of the CNS.10
Children with chronic neuroborreliosis may have nonspecific and only mild clinical signs but concomitant cerebral vascular involvement may be present, implying a risk for possibly severe secondary complications. Additional neurological symptoms together with a differential diagnosis of neuroborreliosis should prompt supplementary radiological evaluation searching for associated cerebral vasculitis.
J Child Neurol. 2015 Aug;30(9):1226-9. doi: 10.1177/0883073814552104. Epub 2014 Oct 14.
Stroke-like Phenomena Revealing Multifocal Cerebral Vasculitis in Pediatric Lyme Neuroborreliosis.
Kurian M1, Pereira VM2, Vargas MI2, Fluss J3.
1 Pediatric Neurology, Pediatric Subspecialties Service, Children's Hospital, Geneva, Switzerland
2 Neuroradiology Unit, University Hospitals, Geneva, Switzerland.
3 Pediatric Neurology, Pediatric Subspecialties Service, Children's Hospital, Geneva, Switzerland.
Stroke-like presentation in Lyme neuroborreliosis is rare in the pediatric age group. We report a previously healthy 12-year-old boy who presented with acute left hemiparesis and meningeal signs. Neuroimaging failed to reveal any cerebral infarction but demonstrated a multifocal cerebral vasculitis involving small, medium and large-sized vessels affecting both the anterior and posterior circulation. Concentric contrast enhancement of the basilar artery was also observed. Further investigations and laboratory findings were consistent with Lyme neuroborreliosis. A rapidly favorable clinical outcome was obtained with appropriate antibiotic treatment along with antiaggregants and steroids. Lyme neuroborreliosis should be considered in the diagnostic differential, not only in adults but also among children, especially in the context of an unexplained cerebral vasculitis.
© The Author(s) 2014.
lyme; neuroborreliosis; pediatric; stroke; stroke-like; vasculitis
PMID: 25316727 DOI: 10.1177/0883073814552104
[PubMed - indexed for MEDLINE]
J Neurol. 2013 Jun;260(6):1569-75. doi: 10.1007/s00415-013-6831-4. Epub 2013 Jan 18.
Neuroborreliosis-associated cerebral vasculitis: long-term outcome and health-related quality of life.
Back T1, Grünig S, Winter Y, Bodechtel U, Guthke K, Khati D, von Kummer R.
1 Department of Neurology and Neurocritical Care, Saxon Hospital Arnsdorf, Hufelandstr. 15, 01477, Arnsdorf/Dresden, Germany.
Neuroborreliosis affects the nervous system after systemic infection with the spirochete Borrelia burgdorferi. Previously, cerebral vasculitis has been regarded as an extremely rare complication of neuroborreliosis. The data on the long-term outcome in patients with cerebral vasculitis due to neuroborreliosis are limited. The objective of this study was to perform a longitudinal analysis of cases of neuroborreliosis-associated cerebral vasculitis. We recruited all patients (n = 11) diagnosed with neuroborreliosis-associated in three neurological departments in an East German region. Inclusion criteria were sudden neurological deficits, magnetic resonance (MR) imaging findings that conform to cerebral ischemia or brain infarction, intrathecal synthesis of borrelia-specific antibodies, and non-atherosclerotic pathology of brain supplying arteries. Vasculitic changes were detected by digital subtraction angiography, MR angiography and/or transcranial Doppler ultrasound. Outcomes were measured by the modified Rankin scale (mRS) and EuroQoL Index. Cerebral vasculitis is a rare complication of Lyme disease (0.3% of all cases in the endemic area). Ten out of 11 patients diagnosed with neuroborreliosis-associated vasculitis cerebral vasculitis using clinical, radiological and immunological criteria developed ischemic stroke or transient ischemic attacks (TIA), 7 patients had recurrent stroke. Vasculitic alterations could be demonstrated in 8 patients that all except one developed ischemic lesions. The median mRS was 3 (range 0-4) at admission and 2 (range 0-6) at discharge. The posterior circulation was affected in 8 of 11 patients; thrombosis of the basilar artery was detected in 2 patients, one died in the acute stage. Neuroborreliosis can cause recurrent stroke or TIA on the basis of cerebral vasculitis. Lumbar puncture is needed for detection of this potentially life-threatening condition. Early recognition and adequate therapy would possibly improve outcome.
PMID: 23329377 DOI: 10.1007/s00415-013-6831-4
[PubMed - indexed for MEDLINE]