Neuroborreliosis-associated cerebral vasculitis: long-term outcome and health-related quality of life

Abstract

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.

Cerebrovascular Events in Lyme Neuroborreliosis

“…9,14,23,28,31 Signs of arachnoiditis, contrast enhancement in the blood vessel wall, and abnormalities of artery caliber observed on MRI support the hypothesis of vasculitis, sometimes confirmed by conventional angiography. 3,10,14,16,22,33,35 Possible radiological findings are both ischemic and hemorrhagic, and nonspecific. However, the multiterritorial aspect of strokes and signs of vasculitis may also be suggestive of a diagnosis of neuroborreliosis.…”

“…5,13,33 Prognosis is mainly associated with the stroke localization as posterior circulation is associated with a poorer prognosis. 3 …”

“…1 Cerebrovascular involvement, and especially stroke, 2 of Lyme disease seems to be a relatively rare complication and estimated to affect from 0.3% to 1% of patients in endemic areas. 3 Since the first reports in 1987, only single or small series case reports can be found in literature and these present a wide range of clinical and radiological features as well as diagnostic criteria, 4 so that studies have concluded it is not worthwhile systematically screening stroke patients for Lyme disease. 5 A more detailed description of signs and symptoms may help physicians to establish a diagnostic strategy to identify neuroborreliosis as the cause of stroke.…”

Lyme neuroborreliosis—epidemiology, diagnosis and management

“…[46][47][48] In Europe, clinical studies have reported signs of polyneuropathy in 40-60% of patients who present with a pathognomonic late cutaneous manifestation of Lyme disease, acrodermatitis chronica atrophicans (ACA). [49][50][51] The ACA-associated polyneuropathy is asymmetric and predominantly sensory, and usually follows the skin lesion.…”

Diplégie faciale révélatrice d’une maladie de Lyme

“…Enfin, au cours de la phase tertiaire de la maladie de Lyme, l'action directe de Borrelia burgdorferi est encore possible, mais les phénomènes dysimmunitaires participent essentiellement à la persistance des lésions neurologiques. Les complications neurologiques peuvent être centrales, sous la forme d'encéphalomyélites progressives (4-10 % des patients atteints de neuroborrélioses), d'accidents vasculaires cérébraux ou de tableaux pouvant mimer une maladie de Horton en rapport avec une vascularite cérébrale (0,3 % des cas), de troubles cognitifs ou encore de neuropathies périphériques [2,5,6,21,22].…”