Stenotic Cerebral Vasculitis in Neuroborreliosis: Prolonged Non-specific Onset Followed by Rapidly Progressing Intracranial Arterial Stenoses Leading to Severe Cerebral Ischemia; Emergency Implantation of Solitaire Stents in the Basilar and Middle Cerebral Arteries with Persistent Mild Neuropsychological Deficit

A 57-year-old patient reported lethargy, vomiting with weight loss, gait disturbance, and rotational dizziness over the 5 months before her recent hospital admission. Despite undergoing an ENT examination, a head CT scan, and a cervical spine MRI, no evident cause for her symptoms was found. She was diagnosed with severe depression and treated with medication. Roughly 3 months following the appearance of these symptoms, a neurological exam revealed gait ataxia and a saccadic pursuit eye movement and subsequent EEG examination indicated a generalized slowing. One week later (January 18), the patient’s family called emergency services following the sudden onset of aphasia, bradykinesia, and confusion. Upon hospital admission to our clinic on January 26, 2023, the patient exhibited aphasia and mild ptosis on the right side, but no paresis. Given MRI findings in line with basal meningitis, the initial differential diagnosis was basal tuberculous meningoencephalitis. Targeted pharmacotherapy was initiated but discontinued when tuberculosis could not be confirmed. IgG and autochthonous antibodies against Borrelia burgdorferi substantiated the diagnosis of stenosing Borrelia burgdorferi-induced vasculitis. The patient was treated with catecholamines to enhance cerebral perfusion, daily methylprednisolone for 7 days, and daily ceftriaxone. Under this regimen, the aphasia and right-sided hemiparesis fluctuated. The National Institutes of Health Stroke Scale (NIHSS) score temporarily improved from 15 to 5 points. On February 5, 2023, the severity of the aphasia increased. On February 6, 2023, right-sided hemiplegia emerged (NIHSS 22). CT perfusion images (CTP) revealed reduced perfusion throughout the entire distribution area of the left middle cerebral artery (MCA) and the posterior circulation. At this point, the patient was receiving daily doses of 1× 100 mg ASA, 1× 20 mg atorvastatin, 3× 750 mg acyclovir, 3× 300 mg isoniazid, 1× 900 mg rifampicin, 1× 750 mg pyrazinamide, and 1× 40 mg prednisolone. The initial cerebrospinal fluid (CSF) examination on January 26, 2023, showed the following (with normal values in brackets): glucose 20 mg/dl [40–75], lactate 3.6 mmol/L [1.2–2.2], total protein 280 mg/dl [15–55], <1000 erythrocytes/μl [<1000], 316 leukocytes/μl [0–5]; 10.6 ASI-B burgdorferi IgG in CSF [<1.5], and 6.5 ASI-B burgdorferi IgM in CSF [<1.5]. Serology: B burgdorferi IgG in serum >240, IgM 4.5. Remaining laboratory results provided no indications of other forms of vasculitis or tuberculosis. On February 6, 2023, the patient’s clinical condition deteriorated. She exhibited moderate right-sided hemiparesis and fluctuating aphasia, which did not improve despite the administration of ceftriaxone and methylprednisolone. Three days later (February 9, 2023), she experienced a further clinical decline characterized by right-sided hemiplegia, global aphasia, leftward gaze deviation, and stupor (NIHSS score of 22). Stenosis of the basilar artery, the left posterior cerebral artery (PCA), the left A1 and M1 segments, and the superior trunk of the left MCA were treated on February 9, 2023, through the implantation of self-expanding Solitaire stents in the basilar artery and left MCA (M1). A localized, clinically insignificant basilar artery dissection occurred during the procedure. In the subsequent months, there was a gradual, incomplete resolution of the hemiplegia and global aphasia. An initial angiographic follow-up 3 months post-stent implantation showed significantly improved posterior and left anterior circulation perfusion despite considerable residual stenoses. Six months after the endovascular procedure, due to hemiparesis and aphasia, the patient’s clinical status corresponded to a modified Rankin Scale (mRS) score of 2. Cerebral vasculitis in neuroborreliosis is the subject of this chapter.