Paraneoplastic Limbic Encephalitis Resembling Acute Herpetic Encephalitis

Introduction. Paraneoplastic limbic encephalitis (PLE) is a rare disorder that typically follows a chronic or subacute course of personality changes, memory loss, seizures, and hallucinations. Early diagnosis is difficult and characteristic symptoms can be mimicked by a variety of conditions. We present a case of PLE, initially presenting as acute herpetic encephalitis.Case Presentation. A 56-year-old male was admitted for evaluation of acute onset headache, fever, and confusion. On neurological examination he was confused with MMSE score of 15/30. CSF analysis revealed marked lymphocytic pleocytosis. A possible diagnosis of acute herpetic encephalitis was rendered and patient was treated with acyclovir. CSF PCR was negative. Cranial MRI revealed bilateral hyperintense lesions in medial temporal lobes with contrast enhancement. Despite treatment with acyclovir patient was deteriorated; thus, a paraneoplastic syndrome was suspected. Chest CT showed a right paratracheal lymph node mass, while a biopsy revealed neuroendocrine lung cancer. Auto antibodies to Hu were also detected. The patient was treated with steroids and chemotherapy. Six months later, he had complete tumour remission and marked neurological improvement.Discussion. PLE can rarely invade acutely, being indistinguishable from herpetic encephalitis. Inclusion of PLE in the differential diagnosis of acute encephalitis is of great clinical significance.

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LGI1 positive paraneoplastic limbic encephalitis: A case report of atypical presentation

Neurology Asia ◽

10.54029/2021auz ◽

2021 ◽

Vol 26 (4) ◽

pp. 865-867

Author(s):

Chien-Chung Cheng ◽

Jia-Ying Sung ◽

Chih-Shan Huang

Keyword(s):

Case Report ◽

Temporal Lobe ◽

Rectal Carcinoma ◽

Medial Temporal Lobe ◽

Limbic Encephalitis ◽

Memory Loss ◽

Atypical Presentation ◽

Paraneoplastic Limbic Encephalitis ◽

Sensory Aphasia ◽

Atypical Presentations

Limbic encephalitis is a rare disorder mainly affecting the medial temporal lobe and is classically paraneoplastic. Autoimmune etiologies also exist, such as antibodies against leucine-rich glioma activated 1 (LGI1). Most cases of anti-LGI1 encephalitis are not associated with tumors. Subacute memory loss is the predominant feature, and most patients develop focal seizures, especially faciobrachial dystonic seizures (FBDSs). Immunotherapies usually show a good response, but are less effective in paraneoplastic cases. We report a case of steroid-responsive anti-LGI1 encephalitis with atypical presentations of sensory aphasia during relapse from rectal carcinoma, an atypical site.

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A Case of Small Cell Lung Cancer Associated with Memory Loss Due to Paraneoplastic Limbic Encephalitis

Haigan ◽

10.2482/haigan.48.202 ◽

2008 ◽

Vol 48 (3) ◽

pp. 202-208 ◽

Cited By ~ 2

Author(s):

Reiko Taki ◽

Sahoko Chiba ◽

Makiko Sugiura ◽

Tsunehiro Sorita ◽

Naoko Saijo ◽

...

Keyword(s):

Lung Cancer ◽

Small Cell Lung Cancer ◽

Cell Lung Cancer ◽

Limbic Encephalitis ◽

Memory Loss ◽

Small Cell ◽

Small Cell Lung ◽

Paraneoplastic Limbic Encephalitis ◽

With Memory

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Anti-NMDAR-Positive Small-Cell Lung Cancer Paraneoplastic Limbic Encephalitis: A Case Report and Literature Review

Case Reports in Neurological Medicine ◽

10.1155/2020/5269352 ◽

2020 ◽

Vol 2020 ◽

pp. 1-5

Author(s):

Raman Sohal ◽

Steven H. Adams ◽

Vishal Phogat ◽

Abha Harish ◽

Carlos Ynigo D. Lopez ◽

...

Keyword(s):

Lung Cancer ◽

Small Cell Lung Cancer ◽

Short Term Memory ◽

Limbic Encephalitis ◽

Memory Loss ◽

Small Cell ◽

Small Cell Lung ◽

Short Term ◽

Term Memory ◽

Paraneoplastic Limbic Encephalitis

Introduction. Paraneoplastic limbic encephalitis (PLE) is a rare disease that presents as rapid onset dementia characterized by short-term memory loss (STM), anxiety, and behavioral changes. Anti-NMDAR antibodies are unfrequently reported in PLE associated with small-cell lung cancer (SCLC). Given that PLE can precede the diagnosis of cancer, it is very important that once infectious, metabolic, nutritional, or structural disorders associated with short-term memory loss are ruled out that vigorous effort must be made to rule out underlying malignancy. Case. We report a rare case of PLE as the presenting symptom of SCLC. A 72-year-old male with history of COPD was brought to the ED by his wife after he was found to have short-term memory loss, including forgetfulness of his wedding anniversary the day before, and anxiety. Neurological exam showed impaired short-term recall on MOCA. CT head showed no evidence of infarct. Lumbar puncture was performed which showed lymphocytic pleocytosis, a nonspecific inflammatory change. CSF panel was negative for HSV, Neisseria, Hemophilus, E. coli, and HIV. Initial EEG was unremarkable, though a repeat EEG showed mild slowing of the posterior dominant rhythm consistent with mild encephalopathy. MRI showed equivocal increased FLAIR on T2-weighted images in the bilateral temporal lobes, left greater than right. CTA thorax showed bulky mediastinal and right hilar LAD. FNA of the R4 lymph node revealed SCLC. The NM bone scan showed no osteoblastic lesions. While the serum autoantibody panel was positive for anti-NMDAR, the CSF autoantibody panel returned entirely negative. Chemotherapy with etoposide and cisplatin was started on Day 4 of admission. The patient’s neurological symptoms showed improvement following chemotherapy. Conclusion. This case highlights the importance of recognizing short-term memory loss as a feature of PLE.

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Paraneoplastic limbic encephalitis mimicking acute herpetic encephalitis

Apollo Medicine ◽

10.4103/am.am_61_19 ◽

2019 ◽

Vol 16 (4) ◽

pp. 240

Author(s):

PushpendraNath Renjen ◽

Charchit Gupta ◽

Dinesh Chaudhari ◽

Anjali Mishra ◽

Shivangi Garg ◽

...

Keyword(s):

Limbic Encephalitis ◽

Paraneoplastic Limbic Encephalitis ◽

Herpetic Encephalitis

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Paraneoplastic Limbic Encephalitis in Hodgkin’s Disease

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100038877 ◽

1996 ◽

Vol 23 (2) ◽

pp. 138-140 ◽

Cited By ~ 27

Author(s):

Sanjeev Deodhare ◽

Paul O’Connor ◽

Danny Ghazarian ◽

Juan M. Bilbao

Keyword(s):

Mental Status ◽

Hodgkin's Disease ◽

Short Term Memory ◽

Limbic Encephalitis ◽

Hodgkin’S Disease ◽

English Literature ◽

Memory Loss ◽

Brain Biopsy ◽

Lymph Node Biopsy ◽

Paraneoplastic Limbic Encephalitis

ABSTRACT:Background: Oat cell carcinoma of the lung is the most common cause of paraneoplastic limbic encephalitis. Association with other malignancies, in particular Hodgkin’s disease, is very rare. Case Report: This 23-year-old male presented with a six month history of progressive alteration in mental status, which consisted of insomnia, short-term memory loss, depression and cognitive impairment. Gadolinium MRI of the head showed intense bilateral contrast enhancement affecting the medial aspects of the temporal lobes in the region of the amygdala and hippocampus. The brain biopsy showed minimal neuronal loss with intense perivascular lymphocytic cuffing and microglia] nodules. Polymerase chain reaction for herpes simplex and cytomegalovirus were negative. With prednisone treatment, the patient’s neurologic status stabilized but did not improve. Four months later, he presented with left axillary lymphadenopathy. Lymph node biopsy was diagnostic of Hodgkin’s disease. During the chemotherapy, his lymphadenopathy subsided and his neurologic and mental status improved. When seen last after completion of his chemotherapy, one year after presentation, he had resumed normal social activities and was enrolled in a university language course. Conclusion: This is the first reported case in the English literature of a biopsy proven paraneoplastic limbic encephalitis associated with Hodgkin’s disease. Hodgkin’s disease should be thought of as a possible cause of paraneoplastic limbic encephalitis in the appropriate clinical setting.

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Paraneoplastic Limbic Encephalitis Secondary To Mixed Non-Seminomatous Germ Cell Tumours

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa161.119 ◽

2020 ◽

Vol 154 (Supplement_1) ◽

pp. S55-S56

Author(s):

A Ullah ◽

S Heneidi ◽

P Biddinger ◽

N Patel ◽

C Wehrle ◽

...

Keyword(s):

Germ Cell ◽

Limbic Encephalitis ◽

Germ Cell Tumors ◽

Testicular Tumor ◽

Primary Malignancy ◽

Rare Presentation ◽

Paraneoplastic Limbic Encephalitis ◽

Nonseminomatous Germ Cell Tumor ◽

Aortic Lymph Node ◽

Alteration Of Consciousness

Abstract Casestudy: Testicular tumors account for 1–2% of all tumors in men, with 95% of these being germ cell tumors. The main risk factor for the development of testicular cancer is cryptorchidism. Paraneoplastic limbic encephalitis is a rare sequela of testicular tumor associated with anti-Ma2 and KLH11 antibodies. The most effective treatment for paraneoplastic limbic encephalitis is treatment of the primary malignancy. We present a 41-year-old male that presented to the emergency department with two weeks of episodic alteration of consciousness and memory disturbances. Negative neurologic evaluation and imaging led to concern for a paraneoplastic process from a distant malignancy. CT imaging revealed an enlarged, necrotic para-aortic lymph node and subsequent ultrasound demonstrated a right sided testicular mass. Right radical orchiectomy was performed. Microscopically, the mass consisted of mixed respiratory epithelium, gastrointestinal glands and squamous epithelium with keratinization consistent with a post-pubertal testicular teratoma with associated in-situ germ cell neoplasia. Resection of the para-aortic mass revealed large anaplastic cells with epithelioid features, nuclear pleomorphism and frequent mitoses. Immunostaining was positive for Pan-Keratin and OCT4, consistent with poorly differentiated embryonal carcinoma. Resection of the primary and metastatic disease, as well as treatment with corticosteroids resulted in resolution of the encephalitis. This presentation of severe neurological disturbances in the setting of a metastatic mixed nonseminomatous germ cell tumor represents a rare presentation of paraneoplastic limbic encephalitis.

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A Rare Case of Spontaneous Arachnoid Cyst Rupture Presenting as Right Hemiplegia and Expressive Aphasia in a Pediatric Patient

Children ◽

10.3390/children8020078 ◽

2021 ◽

Vol 8 (2) ◽

pp. 78

Author(s):

Anne Bryden ◽

Natalie Majors ◽

Vinay Puri ◽

Thomas Moriarty

Keyword(s):

Cognitive Processing ◽

Arachnoid Cyst ◽

Memory Loss ◽

Acute Onset ◽

Word Finding ◽

Outpatient Visits ◽

The Family ◽

History Of ◽

Cyst Rupture ◽

The Brain

This study examines an 11-year-old boy with a known history of a large previously asymptomatic arachnoid cyst (AC) presenting with acute onset of right facial droop, hemiplegia, and expressive aphasia. Shortly after arrival to the emergency department, the patient exhibited complete resolution of right-sided hemiplegia but developed headache and had persistent word-finding difficulties. Prior to symptom onset while in class at school, there was an absence of reported jerking movements, headache, photophobia, fever, or trauma. At the time of neurology consultation, the physical exam showed mildly delayed cognitive processing but was otherwise unremarkable. The patient underwent MRI scanning of the brain, which revealed left convexity subdural hematohygroma and perirolandic cortex edema resulting from ruptured left frontoparietal AC. He was evaluated by neurosurgery and managed expectantly. He recovered uneventfully and was discharged two days after presentation remaining asymptomatic on subsequent outpatient visits. The family express concerns regarding increased anxiety and mild memory loss since hospitalization.

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