Table 8.

Cases of cerebral vasculitis secondary to Bartonella infection

Cerebral vasculitis associated with Bartonella infection
Diagnosis	Age/ sex	Medical history	Neurological symptoms	Pertinent diagnostics	Bartonella serology/ PCR	Treatment/ duration	Outcome	Ref.
Cerebral vasculitis	60/F	3 weeks thunderclap headaches, photo and phonophobia, nausea and vomiting	Brief episode of slurred speech, expressive aphasia, right facial droop and right hemi-paresis	Initial brain CT/MRI NSF. DSA: intracranial med. + lg. vessel narrowing + fusiform dilatations. IVWI: multifocal concentric vessel wall thickening + enhancement1	Pending	Empiric high dose IV steroids	Resolution of all symptoms	[143]
					Bartonella IgM 1:256 IgG neg	Doxycycline + rifampin + oral steroids	4-week follow-up MRI improvement. Decreasing titers (IgM 1:80)
Cerebral vasculitis and infarction	11/F	Flu-like illness night sweats, abdominal pain, bloating and constipation	Sudden onset headaches, difficulty walking, ataxia and left-sided paraparesis	MRI: large, focal demyelinating mass right parietal lobe. Biopsy: vasculitis, cerebral infarction2	Not initially tested	High dose IV steroids for presumptive autoimmune disease	Multiple ensuing diagnoses; idiopathic vasculitis, GBS, MS, ADEM over 3 years	[144]
		Non-febrile respiratory illness, chest pain	3 weeks later headaches anxiety, ocular floaters, depression, fatigue, visual and auditory hallucinations	No additional diagnostics noted		No additional treatment noted	1-year period of time elapsed to neurocognitive signs
		No other episodic fever or other signs noted	Neurocognitive dysfunction, left-sided paralysis, hemianopia, seizures, dysphagia, laryngitis and severe confusion	No additional diagnostics noted		IVIG	Recovery over several months; new allergic reactions to pork, lactose, gluten and corn
		No other inciting episodes noted	Recurrent progressive paresis 5 years after last episode	IgA, IgM, IgG low		IVIG, fluconazole	Status epilepticus requiring medically induced coma
			Tetraplegia, dysphasia, severe facial palsy upon coma recovery	Equivocal Lyme on Western blot		Penicillin added to treatment plan above	Rapid improvement in all neurological signs
			Overall deterioration requiring physical support and full-time care3	Bh visualized in FFPE brain tissue	IFA neg Bh + PCR/ sequence4 Repeat Bh testing neg5	Atovaquone, azithromycin + ceftriaxone	Dramatic improvement in all clinical signs
						Ceftriaxone, metronidazole, azithromycin × 9 weeks	Recurrent seizure and cognitive decline then slow improvement over 7 months

¹CSF and transcranial Doppler study NSF. Infectious and inflammatory screening neg

²Surrounding perivascular lymphoplasmacytic vascular infiltration + sparse hemosiderin in small arteries and venules. Perivascular lymphocytes, primarily T cells, scattered B cells. MIB1 minimal, no EBV immunoreactivity

³Neurobartonellosis, babesiosis and Lyme suspected based on clinical presentation

⁴IFA for Bh strains San Antonio 2 and Houston 1, B. vinsonii subsp. Berkhoffii genotypes I–III, B. koehlerae all negative. Positive PCR and genetic sequencing for Bh (99% identity NCBI) from blood. PCR + from FFPE brain tissue

⁵Negative IFA and PCR as above, from blood, serum and post-BAPGM enrichment blood culture

CT computerized tomography, MRI magnetic resonance imaging, NSF no significant findings, DSA digital subtraction angiography, IVWI intracranial vessel wall imaging, IV intravenous, MIB1 Mindbomb E3 ubiquitin protein ligase 1 minimal staining, Epstein-Barr virus negative, GBS Guillain-Barré syndrome, MS multiple sclerosis, ADEM acute disseminated encephalomyelitis, IVIG intravenous immunoglobulin, Ig immunoglobulin, Bh Bartonella henselae, FFPE formalin-fixed paraffin embedded tissue, Bh Bartonella henselae, IFA immunofluorescent antibody, PCR polymerase chain reaction