Amyotrophic Lateral Sclerosis As A Paraneoplastic Manifestation in the Neuroendocrine Tumor of Stomach: A Case Report
Motor neuron diseases have been reported as a rare paraneoplastic syndrome (PNS) of a systemic neoplasm. We present a patient with amyotrophic lateral sclerosis (ALS) in association with neuroendocrine tumor (NET) of stomach, which is the first case of motor neuronopathy with underlying neuroendocrine tumor. A 79-year old woman presented with a two months history of progressive dysphagia, spastic dysarthria and marked fasciculation in her atrophic tongue. Gag reflexes were diminished bilaterally. Other cranial nerves were intact. In muscle testing there was significant atrophy in thenar and hypothenar areas of both hands compatible with diffuse motor neuronopathy with active denervation. Upper GI endoscopic study showed patchy erythematous mucosa with congestion in body of stomach, Histological biopsy of stomach confirmed the neuroendocrine tumor (NET). The importance of considering a paraneoplastic syndrome in a patient with presentation of ALS, which can leads to searching for underlying neoplasm before its apparent signs and symptoms, to initiate tumor treatment so much sooner. In addition even though paraneoplastic motor neuron disease is rare, treating the underlying neoplasm may resolve neurologic signs and symptoms.
Gordon PH, Rowland LP, Younger DS, Sherman WH, Hays AP, Louis ED, Lange DJ, Trojaborg W, Lovelace RE, Murphy PL, Latov N. Lymphoprolifrative disorders and motor neuron disease: an update.Neurology 1997;48(6):1671-8.
Forsyth PA, Dalmau J, Graus F, Cwik V, Rosenblum MK, Posner JB. Motor neuron syndrome in cancer patients. AnnNeurol 1997;41(6):722-30.
Dropcho EJ. Neurological paraneoplastic syndromes. J Neurol Sci 1998;153(2):264-78.
Khealani B.A, Quershi R, Wasay M. Motor neuronopathy associated with adenocarcinoma of esophagus. J Pak Med Assoc 2004;54(3):165-6.
Mehmet H, Ozet A, Kuzhan O, Komurcu F, Arpaci F, Ozturk B, Ataergin S. Paraneoplastic motor neuron disease resembling amyotrophic lateral sclerosis in patients with renal cell carcinoma. International journal of the Kuwait university Health Science Centre 2009;18(1):73-5.
Evans BK, Fagan C, Arnold T, Dropcho EJ, Oh SJ. Paraneoplastic motor neuron disease and renal cell carcinoma improvement after nephrectomy 1990;40(10):1638.
Anderlini,P.,Buzaid,A.C,Legha,S.,Acute rhabdomyolisis aftre a metatatic melanoma,Cancer 1995;(76):678-679
Tanaka,K,.Tanaka,M,.Inuzuka,T.,et al.Cytotoxic T lymphocyte mediated cell death in paraneoplastic sensory neuronopathy with anti-Hu antibody,J neurol Sci,1999;(163):159-162.
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