<i>Borrelia burgdorferi</i>-Seropositive Chronic Encephalomyelopathy: Lyme Neuroborreliosis?

Abstract

A 36-year-old Japanese woman presented with progressive cerebellar signs and mental deterioration of subacute course after her return from the USA. Her serum antibody to spirochete Borrelia burgdorferi was significantly elevated. A necropsy 4 years after her initial neurological signs revealed multifocal inflammatory change in the cerebral cortex, thalamus, superior colliculus, dentate nucleus, inferior olivary nucleus and spinal cord. The lesions showed spongiform change, neuronal cell loss, astrocytosis and proliferation of activated microglial cells. The internal capsule was partially vacuolated and the spinal cord, notably at the thoracic level, was demyelinated and cavitated in the lateral funiculus. Microglial cells aggregated within and around the spongiform lesions and microglial nodules were present in the medulla oblongata. Use of Warthin-Starry stain demonstrated silver-impregnated organisms strongly suggesting B. burgdorferi in the central nervous tissues. The dentate nucleus and inferior olivary nucleus showed the most advanced lesions with profound fibrillary gliosis. Occlusive vascular change was relatively mild, and fibrous thickening of the leptomeninges with lymphocyte infiltrates was localized in the basal midbrain. The ataxic symptoms were due to the dentate and olivary nucleus lesions and mental deterioration was attributable to the cortical and thalamic lesions. Spongiform change, neuronal cell loss, and microglial activation are characteristic pathological features in the present case. The cerebellar ataxia and subsequent mental deterioration are unusual clinical features of Lyme neuroborreliosis. Spirochete B. burgdorferi can cause focal inflammatory parenchymal change in the central nervous tissues and the present case may be an encephalitic form of Lyme neuroborreliosis.

Opsoclonus myoclonus syndrome in two cases with neuroborreliosis

“…The combination of OMS and neuroborreliosis has been reported previously, in a child [5] and in an autopsy study [6]. In both our patients and the child, only IgM antibodies in CSF were detected.…”

Adult-onset opsoclonus-myoclonus-ataxia syndrome as a manifestation of brazilian lyme disease-like syndrome: a case report and review of literature

“…In our case, the MRI showed a tenuous and unspecific thalamic hyper-signal in T2-weighted and FLAIR sequences. Even though the same findings had been described in one idiopathic case by Bataller et al,15 Kobayashi et al33 found thalamic lesions in the autopsy of a Borrelia burgdorferi -seropositive chronic encephalomyelopathy. Furthermore, blood and CSF tests are most useful in diagnosing an infection, but do not confirm or exclude a paraneoplastic or autoimmune etiology.…”

Localization of Borrelia Burgdorferi in the Nervous System and Other Organs in a Nonhuman Primate Model of Lyme Disease

“…The most common manifestations of Lyme neuroborreliosis are headache, meningismus, or radiculopathic symptoms, whereas CNS parenchymal involvement is rare (Hansen and Lebech, 1992;Pacher et al, 1998a). The localization of spirochetes in the CNS of humans has been reported only in a handful of cases using nonspecific silver impregnation techniques (Kobayashi et al, 1997;Kuntzer et al, 1991;Miklossy et al, 1990). The positivity of the PCR-ELISA in samples from the cerebrum and brainstem likely represents leptomeningeal, rather than parenchymal, localization.…”