Case Report - Volume 2 - Issue 4

Anti-Hu Encephalitis Associated With Small Cell Lung Cancer,A Case Report

David Symeonidis1*; Theodora-Maria Papadimitriou2; Evridiki Mazlimoglou2; Spyridon Xynogalos3

1Resident of Oncology, Metaxa Cancer Institute of Piraeus, Greece.
2Resident of Internal Medicine, Metaxa Cancer Institute of Piraeus, Greece
3Head of Oncology Department, Metaxa Cancer Institute of Piraeus, Greece.

Received Date : June 18, 2022
Accepted Date : July 27, 2022
Published Date: Aug 17, 2022
Copyright:© David Symeonidis 2022

*Corresponding Author : David Symeonidis, Resident of Oncology,Metaxa Cancer Institute of Piraeus, Greece
Email: davidsymeonidis@yahoo.com
DOI: Doi.org/10.55920/2771-019X/1216

Keywords

Encephalitis; anti-Hu; SCLC.

Introduction

Paraneoplastic limbic encephalitis (PLE) is a rare neurologic that can be a manifestation of lung cancer. These manifestations are frequently seen in patients diagnosed with small cell lung cancer (SCLC), as in our case. This is a case report of a 66-years-old woman, with SCLC that has been diagnosed due to paraneoplastic encephalitis staining positive for anti-Hu antibodies. Sometimes differential diagnosis involves endocrinopathies and other neurological conditions, so many specialists must co-operate in each patient

Case Report

A 66-year-old woman, with personal history of dyslipidemia and smoking 40 pack- years, was presented to our hospital’s emergency department with tinnitus on the right side, dizziness, walking instability, hoarseness and progressively worsening blurred vision. She was examined by a neurologist, an ophthalmologist, and an otolaryngologist. The clinical examination of the neurologist revealed horizontal and vertical nystagmus, pupils with anisocoria, mydriasis on the left pupil, non-react to the light on both sides, eyelid drop on the left eye, diplopia on the left eye, strabismus on the right eye with esotropia, and paralysis on the right half face. There was no Babinski sign on both feet and cerebellum tests revealed only walking instability. The examination from the ophthalmologist was negative for significant clinical evidence and the bythoscopy was clear. CT scan of the brain did not provide us any important information, it was a clear tomography. Afterwards, a Magnetic Resonance (MRI) was also negative for new evidence. Then, the patient underwent on lumbar puncture, twice, that was negative for infection. The cytological examination of the cerebrospinal fluid was negative for malignant cells. Subsequently, a CT scan of the thorax and the abdomen revealed a tumor in the middle mediastinum in front of the right portal. Due to the findings, the patient undergone endobronchial ultrasound and fine needle aspiration that was positive for small cell lung cancer. In addition, onconeural antibody tests was sent. The tests revealed positive anti-Hu antibodies and confirmed the paraneoplastic anti-Hu encephalitis. During her hospitalization, the patient was clinically deteriorated and was put in high dose γ-globin therapy for 5 days (25gr/24hours) and complementary therapy with methylprednisolone for 5 days (1gr/24hours). The treatment did not have the desired result, therefore the patient referred to our oncology department for further treatment. To our hospital, the patient started immediately chemotherapy with carboplatin AUC5 and etoposide (100mg/m2 ) for 3 days. After the first cycle of chemotherapy, the patient had rapid clinical improvement and after 3 cycles of chemotherapy, the tumor has shrinkage, and we are now planning to add immunotherapy.

Figure: We can see here strabismus and gaze dedication.

Discussion

Paraneoplastic limbic encephalitis accounts for about 20% of all neurological cancer- related complications [1] and had been most associated with SCLC [2]. In many patients, this neurologic disorder develops before the cancer is clinically found a situation that is similar to our case study [3]. Paraneoplastic cases are associated with antibodies directed against antigens expressed by both the tumor and the nervous system [4]. These are called anti-Hu and can be found in 50% of the cases. Recent studies report that paraneoplastic encephalitis with anti-Hu antibodies mostly is resistant to immunosuppressive therapies, so these antibodies may serve not only as a diagnostic indicator, but also as a predictive marker for treatment response. There are some cases (15%) with anti-Hu encephalitis free of any type of neoplasia and there also are observations in pediatric population, associated with neuroblastoma or opsoclonus-myoclonus syndrome [5].

The clinical picture may differ between encephalitis, myelitis, or a combination, depends on the anatomic site affected [6]. The temporal lobes, the brainstem, the cerebellum, and the dorsal roots are the most frequent sites while, the clinical manifestations correlate with these anatomic sites are varied for example patients presenting with sensory neuropathy when the dorsal roots are involved or with memory issues, seizures, or behavioral changes when the temporal lobes are affected [7, 8].

A large case series included 200 cases with anti-Hu encephalitis, showed that the appropriate treatment is associated the ameliorations of the symptoms and stabilization of the whole neurological situation of the patients [5]. In addition to this there was observed decreased mortality. Other reports support the use of immunoglobulin, corticosteroids, plasma exchange and human chorionic gonadotropin. The prognosis is poor when vital structures are involved, however the outcome may improve if there is tumor shrinkage as a treatment result [9-11].

Conclusions

Anti-Hu encephalitis is a rare neurological situation most frequently associated with SCLC. The clinical behavior involves changes in behavior, loss of memory, seizures, myoclonus and visibility symptoms, not otherwise specified. The timely diagnosis is very important, because as soon as the treatment began, then there are more possibilities for the patient symptoms to alleviate.

Summary

Paraneoplastic limbic encephalitis (PLE) is a rare neurologic that can be a manifestation of lung cancer. These manifestations are frequently seen in patients diagnosed with small cell lung cancer (SCLC), as in our case. This is a case report of a 66-year-old woman, with personal history of dyslipidemia and smoking 40 pack- years, was presented to our hospital’s emergency department with tinnitus on the right side, dizziness, walking instability, hoarseness and progressively worsening blurred vision. A CT scan of the thorax and the abdomen revealed a tumor in the middle mediastinum in front of the right portal. Due to the findings, the patient undergone endobronchial ultrasound and fine needle aspiration that was positive for small cell lung cancer. In addition, onconeural antibody tests was sent. The tests revealed positive anti-Hu antibodies and confirmed the paraneoplastic anti-Hu encephalitis. To our hospital, the patient started immediately chemotherapy with carboplatin AUC5 and etoposide (100mg/m2 ) for 3 days. After the first cycle of chemotherapy, the patient had rapid clinical improvement and after 3 cycles of chemotherapy, the tumor has shrinkage, and we are now planning to add immunotherapy.

Conflict of Interests: NO.

References

  1. GultekinSH,RosenfeldMR,VoltzR,EichenJ,PosnerJB,DalmauJ. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000 Jul; 123(7): 1481-94.
  2. Saiz A, Dalmau J, Butler MH, Chen Q, Delattre JY, De Camilli P, et al. Anti-amphiphysin I antibodies in patients with paraneoplastic neurological disorders associated with small cell lung carcinoma. J Neurol Neurosurg Psychiatry. 1999 Feb; 66(2): 214-7.
  3. HonnoratJ,AntoineJC.Paraneoplasticneurologicalsyndromes. Orphanet J Rare Dis. 2007 May; 2(1): 22.
  4. Graus F, Dalmau J. Paraneoplastic neurological syndromes: diagnosis and treatment. Curr Opin Neurol. 2007 Dec; 20(6): 732–7.
  5. Graus F, Keime-Guibert F, Reñe R, et al.: Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain. 2001; 124: 1138-1148. DOI:10.1093/brain/124.6.1138
  6. Dalmau J, Graus F, Rosenblum MK, Posner JB: Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients. Medicine. 1992; 71: 59-72.
  7. Sillevis Smitt P, Grefkens J, de Leeuw B, van den Bent M, van Putten W, Hooijkaas H, Vecht C: Survival and outcome in 73 anti- Hu positive patients with paraneoplastic encephalomyelitis/sensory neuronopathy. J Neurol. 2002; 249: 745-753.
  8. Saiz A, Bruna J, Stourac P, et al.: Anti-Hu-associated brainstem encephalitis. J Neurol Neurosurg Psychiatry. 2009; 80: 404-407.
  9. Dalmau J, Graus F, Rosenblum MK, Posner JB: Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients. Medicine. 1992; 71: 59-72.
  10. Van Broekhoven F, de Graaf MT, Bromberg JE, et al.: Human chorionic gonadotropin, treatment of anti-Hu-associated paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2010; 81: 1341-1344.
  11. Darnell RB, DeAngelis LM: Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies. Lancet. 1993; 341: 21-22.
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