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 »  Introduction
 »  Case report
 »  Discussion
 »  References

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Year : 2001  |  Volume : 49  |  Issue : 2  |  Page : 185-7

Paraneoplastic limbic encephalitis associated with bronchogenic carcinoma : a case report.


Department of Neurology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, 190 011, India.

Correspondence Address:
Department of Neurology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, 190 011, India.

  »  Abstract

Paraneoplastic limbic encephalitis is a rare clinical entity, associated most often with the oat cell carcinoma of the lung. Clinically, it presents with affective changes in personality, memory loss, confusional state, hallucinations, and seizures; with dementia being the common feature as the disorder progresses. Response to treatment is disappointingly poor.

How to cite this article:
Wani M A, Dar J A, Khan M A, Rehman A. Paraneoplastic limbic encephalitis associated with bronchogenic carcinoma : a case report. Neurol India 2001;49:185


How to cite this URL:
Wani M A, Dar J A, Khan M A, Rehman A. Paraneoplastic limbic encephalitis associated with bronchogenic carcinoma : a case report. Neurol India [serial online] 2001 [cited 2020 Oct 27];49:185. Available from: https://www.neurologyindia.com/text.asp?2001/49/2/185/1267




   »   Introduction Top

The occurrence of encephalomyelitic changes in association with carcinoma has been described by several authors, being associated in most of the cases with oat cell carcinoma of the lung. The pathologic changes of extensive nerve cell loss with necrosis, microglial proliferation and perivascular lymphocytic infiltration occur diffusely throughout the brain and spinal cord, but more often predominate in the limbic lobes ('limbic encephalitis').[1] Clinically, patients with limbic encephalitis present with affective changes in personality, selective early memory loss suggestive of korsakoff psychosis, or confusion and hallucinations.[2] In some cases, the initial presentation is an amnesic syndrome. Dementia becomes common as the disorder progresses.[3] We present a case report of this rare condition associated with bronchial malignancy.


   »   Case report Top

A 53 years old man presented with agitated behaviour, drowsiness, irrelevant talking, confusion, hallucinations and loss of memory. Eight days prior to hospital admission, he had multiple generalised tonic clonic seizures and weakness of right side of body, which improved subsequently. There was no significant past medical or family history. He was a smoker and nonalcoholic. Physical examination revealed normal respiratory, cardiovascular or gastrointestinal systems. Patient was conscious, incoherent, agitated talking irrelevant and was dysarthric. He could only recognise his family members and remembered nothing else. He had mild right sided hemipareisis.
Routine blood tests were normal initially, (later on developed normochromic normocytic anaemia). A chest radiograph demonstrated a mass in the left hilar region [Figure - 1]. On bronchoscopy, a stenosing growth was seen in left upper apicoposterior segmental bronchous. EEG showed predominant fast beta activity along with excessive slow wave discharges. CT scan was normal. CSF revealed 8 cells/cumm, mostly lymphocytes and no malignant cells. Other CSF parameters including protein, globulin, glucose, chloride and smears were normal. Cranial MRI revealed bilateral symmetrical hyperintensities on T2WI and flair image seen in medial temporal lobes and parahippocampal gyri [Figure - 2]. They were minimally hypointense on T1WI image with no foci of haemorrhages seen in it. The patient's mental state progressively deteriorated for 7 weeks and he later died of aspiration pneumonia.


   »   Discussion Top

Although, we do not have pathological confirmation of limbic encephalitis in our patient, the combination of clinical picture, involvement of medial temporal lobes as shown by MRI, EEG abnormality, abnormal chest X-ray and exclusion of other causes made the diagnosis of limbic encephalitis very likely. Limbic encephalitis is usually associated with small cell lung cancer[4] and may occasionally be associated with thymoma,[5],[6] testicular,[7],[8] bladder,[9] colon[10] and kidney malignancy[11] or with Hodgkin's disease.[6]
Limbic encephalitis is characterised by subacute and severe neurological disorder, consisting of mental confusion, memory impairment, cognitive dysfunction, hallucinations, depression, personality changes and sleep disturbances, occurring in varying combinations in more than 90% of patients.[12] Duyckaerts et al[13] and Gascon and Gilles[14] established the correlation between bilateral destruction of limbic neurons, behavioural changes and recent memory deficits in patients with limbic dementia.
Cerebrospinal fluid may be normal or show mild elevation of proteins, pleocytosis and positive oligoclonal bands.[4] MR usually reveals focal involvement of one or both temporal lobes and typical lesions on MRI consist of increased signal on T2WI or atrophy on T1WI in the medial aspect of one or both temporal lobes.[15] EEG is often normal or may show non-specific generalised theta activity depending upon the stage of disease. The present case showed pleocytosis in CSF with abnormal findings on MRI and excessive slow waves in EEG. Anti-Hu antibodies are considered a marker for neurological paraneoplastic syndrome associated with small cell lung cancer[11] but frequency of anti-Hu antibodies varies among different paraneoplastic syndromes. Alamowitch et al[4] were able to find them in only 50% of the patients with limbic encephalitis in their series of 16 patients. Moreover, they found no difference in the clinical and radiological features of the disease between anti-Hu +ve and anti-Hu -ve patients. We were not able to test our patient for anti-Hu status because of the non-availability of facility at our institute.
Although, the remission of various neurological paraneoplastic syndromes is known to follow treatment of primary tumour,[16] the effect on paraneoplastic limbic encephalitis is disappointing, with only one reported case of histologically proven limbic encephalitis associated with testicular carcinoma[17] responding to orchidectomy and chemotherapy. The pathogenesis of limbic encephalitis is not clear but a slow viral infection has been speculated with equal evidence against it. Alternatively, an immune damage of limbic neurons has been found to be more plausible explanation for paraneoplastic limbic encephalitis.[12]

 

  »   References Top

1.Adams RD, Victor M : Encephalomyelitis associated with carcinoma. In : Principles of neurology 6th ed. McGraw Hill Inc. Health Profession division. 1997; 687-688.   Back to cited text no. 1    
2.Newman NJ, Bell IR, Mckee AC : Paraneoplastic limbic encephalitis. Neuropsychiatric presentation (Review). Biol Psychiatry1990; 27 : 529-542.   Back to cited text no. 2    
3.Robert H, Brown JR : Paraneoplastic neurological syndromes. In : Harrison's principles of internal medicine, Isselbacher, Braunwald Wilson et al McGraw Hill Inc. 13th ed. 1994; 1878-1882.   Back to cited text no. 3    
4.Alamowitch S, Graus F, Uchuya M et al : Limbic encephalitis and small cell lung cancer, clinical and immunological features. Brain1997; 120 : 923-928.   Back to cited text no. 4    
5.Antoine JC, Honnorat J, Anterion CT et al : Limbic encephalitis and immunological perturbations in two patients with thymoma. J Neurol Neurosurg Psychiatry 1995; 58 : 706-710.   Back to cited text no. 5    
6.Ingenito GG, Berger JR, David NJ et al : Limbic encephalitis associated with thymoma. Neurology 1990; 40 : 382.   Back to cited text no. 6    
7.Ahern Cl, O'Connor M, Dalmau J et al : Paraneoplastic temporal lobe epilepsy with testicular neoplasia and atypical amnesia. Neurology1994; 44 : 1270-1274.   Back to cited text no. 7    
8.Burton GV, Bullard DE, Walther PG et al : Paraneoplastic limbic encephalopathy with testicular carcinoma. A reversible neurological syndrome. Cancer 1988; 62 : 2248-2251.   Back to cited text no. 8    
9.Case records of Massachusetts general hospital weekly clinicopathological exercises cases 30-1985. A 52 year old woman with progressive neurological disorder and a pelvic mass. N Engl J Med 1985; 313 : 249-257.   Back to cited text no. 9    
10.Tsukamoto T, Mochizuki R, Mochizuki N et al : Paraneoplastic cerebellar degeneration and limbic encephalitis in a patient with adenocarcinoma of colon. J Neurol Neurosurg Psychiatry1993; 56 : 713-716.   Back to cited text no. 10    
11.Dalmau J, Graus F, Rosenblum MK et al : Anti Huassociated paraneoplastic encephalomyelitis / sensory neuronopathy, a clinical study of 71 patients. Medicine (Baltimore) 1992; 71 : 59-72.   Back to cited text no. 11    
12.Bakheit AMO, Kennedy PGE, Behan PO : Paraneoplastic limbic encephalitis. Clinicopathological correlations. J Neurol Neurosurg Psychiatry1990; 53 : 1084-1088.   Back to cited text no. 12    
13.Duyckaerts C, Derouesne C, Signoret JL et al : Bilateral and limited amygdalohippocampal lesions causing pure amnesic syndrome. Ann Neurol 1985; 18 : 314-319.   Back to cited text no. 13    
14.Gascon GG, Gilles F : Limbic dementia. J Neurol Neurosurg Psychiatry1973; 6 : 421-430.   Back to cited text no. 14    
15.Dirr Ly, Elster AD, Donofrio PD et al : Evolution of brain MRI abnormalities in limbic encephalitis. Neurology 1990; 40 : 1304-1306.   Back to cited text no. 15    
16.Brennan LV, Craddock PR : Limbic encephalopathy as a non-metastatic complication of oat cell lung cancer. Am J Med 1983; 75 : 518-520.   Back to cited text no. 16    
17.Markhan M, Abeloff MD : Small lung cancer and limbic encephalitis. Ann Int Med 1982; 26 : 785.   Back to cited text no. 17    

 

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