Autoimmune Paraneoplastic Limbic Encephalitis (PLE) and
Paraneoplastic Cerebellar Degeneration (PCD) in a Patient with an Invasive Thymoma
Kettnerová Z., Vlachová I., Herzig R., Koranda S., Burešová J., Žondra B., Buřval S. , Urbánek K., Tichý T.
Neurologická klinika LF a FN, Olomouc, Klinika nukleární medicíny LF a FN, Olomouc, Radiologická klinika LF a FN, Olomouc, Ústav patologie LF UP, Olomouc |
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Summary:
The authors describe the case of a 64-year-old man with myasthenia gravis who developed only several
weeks after removal of an invasive thymoma paroxysms of vegetative dysregulation with tachypnoea
and tachycardia initiated by cacosmias cumulating in epileptic states, followed by states of derealiza-
tion with anxiety, subsequently with important disorders of recent memory and progressing by uneven
deterioration of the intellect. In the late stage of the disease cerebellar symptomatology progressed
with nystagmus and severe organic psychosyndrome. EEG revealed repeatedly signs of increased
paroxysmal tendency with sharp bitemporal waves in zone alpha, more on the left. CT of the brain was
repeatedly negative, on the MRI of the brain there was a small hypersignal focus in the cerebellum on
the right. Single-photon emission computed tomography (SPECT) of the brain revealed a relatively
lower perfusion of basal parts of the left temporal lobe, in cerebrospinal fluid there was only a non-spe-
cific increase of the total protein level. The coincidence of clinical manifestati ons and malignant tumour
made the authors consider the diagnosis of limbic paraneoplastic autoimmune encephalitis. This
suspicion was supported by immunological examination. Cyclosporin A treatment led to marked transient remission of the disease. After more than four years despite subsequent treatment by
plasmapheresis and immunoglobulins, the condition led rapidly to a fatal outcome. The post-mortem
finding confirmed paraneoplastic limbic encephalopathy incl. signs of paraneoplastic cerebellar degeneration. The authors mention the problem and variety of the clinical picture of paraneoplastic
affliction of the central nervous system (CNS) incl. possible therapy.
Key words:
paraneoplastic syndrome, limbic encephalitis, paraneoplastic cerebellar degeneration
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