|
Title
|
|
|
|
Long lasting trigeminal neuropathy, limbic encephalitis and abdominal ganglionitis without primary cancer : an atypical case of Hu-antibody syndrome
| |
|
Author
|
|
|
|
| |
|
Abstract
|
|
|
|
| Objectives: Anti-Hu antibodies (Hu-Abs) are the most frequent onconeural antibodies associated with paraneoplastic neurologic syndromes (PNS). PNS include a variety of neurological syndromes, affecting less than 1/10,000 patients with cancer. In the majority of cases, PNS will manifest before the malignancy is diagnosed. We found a case in which PNS was diagnosed without finding a primary malignancy after extensive work-up and even post-mortem autopsy. Patient and methods: We present a case report of a 58-year-old man. This article includes extensive clinical work-up, full-body autopsy and brain autopsy with classical histochemical and myelin stainings and immunohistochemistry was performed. Results: The patient developed a progressive trigeminal neuropathy over a period of 5 years, in combination with cerebellar degeneration, asymmetrical brainstem and limbic encephalitis. Serum showed repeatedly high anti-Hu antibodies. Comprehensive cancer screening could not demonstrate any primary malignancy. Therapy with corticosteroids, plasma exchange, cyclophosphamide and rituximab showed no beneficial effect. He died from the complications of enteric ganglionitis 5 years after onset of the first symptoms. A postmortem autopsy could not detect a primary malignancy either. Brain morphology is described in detail. Conclusion: Paraneoplastic anti-Hu encephalitis cases associated with SCLC or other primary neoplasms are well known. An adult with a progressive multifocal neurological syndrome in the presence of positive anti-Hu antibodies, but without any primary neoplasm after a follow-up over 5 years is unusual. |
| |
|
Language
|
|
|
|
English
| |
|
Source (journal)
|
|
|
|
Clinical neurology and neurosurgery / Netherlands Society of Neurology. - Assen, 1974, currens
| |
|
Publication
|
|
|
|
Assen
:
Van Gorcum
,
2020
| |
|
ISSN
|
|
|
|
0303-8467
[print]
1872-6968
[online]
| |
|
DOI
|
|
|
|
10.1016/J.CLINEURO.2020.105849
| |
|
Volume/pages
|
|
|
|
194
(2020)
, p. 1-4
| |
|
Article Reference
|
|
|
|
105849
| |
|
ISI
|
|
|
|
000540254700038
| |
|
Pubmed ID
|
|
|
|
32388246
| |
|
Medium
|
|
|
|
E-only publicatie
| |
|
Full text (Publisher's DOI)
|
|
|
|
| |
|
Full text (publisher's version - intranet only)
|
|
|
|
| |
|