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  Česky / Czech version Čes. a Slov. Neurol. Neurochir., 64/97, 2001, No. 3, p. 182-187
 
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
 


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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