Paraneoplastic Neurologic Syndrome: A Report of Two Patients

We encountered two patients with paraneoplastic neurologic syndrome who visited our ENT clinic complaining of vertigo. The first patient was a 73-year-old woman who was admitted to our hospital because of dysequilibrium and dysarthria of subacute onset. Chest CT showed swelling of the mediastinal an...

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Published inEquilibrium Research Vol. 60; no. 1; pp. 16 - 23
Main Authors Kinoshita, Masanobu, Osanai, Ryuichi, Tsubaki, Keiju, Namiki, Satoko
Format Journal Article
LanguageEnglish
Japanese
Published Japan Society for Equilibrium Research 2001
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ISSN0385-5716
1882-577X
DOI10.3757/jser.60.16

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Abstract We encountered two patients with paraneoplastic neurologic syndrome who visited our ENT clinic complaining of vertigo. The first patient was a 73-year-old woman who was admitted to our hospital because of dysequilibrium and dysarthria of subacute onset. Chest CT showed swelling of the mediastinal and right hilar lymphnodes. Pathological examination of the hilar lymphnodes taken by thoracotomy revealed small cell carcinoma of the lung. Brain CT and brain MRI were normal. Cytological study of the cerebrospinal fluid was class I. Paraneoplastic cerebellar degeneration was diagnosed. Neuro-otological examinations revealed: 1) gaze nystagmus on both right and left lateral gaze, 2) saccadic pursuit, 3) hypometric saccades, 4) diminished caloric nystagmus and 5) decreased visual suppression of caloric nystagmus. Treatment with plasmapheresis was successful to improve limb/truncal ataxia, dysarthria, and neuro-otological findings. The second patient was a 54-year-old woman who had had surgical treatment 3 years before for ovarian cancer. She visited our ENT clinic because of vigorous vertigo and diplopia of sudden onset. Neurological examinations revealed reduced deep tendon reflexes and neuro-otological examinations revealed: 1) bilateral abducens nerve palsy, 2) gaze nystagmus on both right and left lateral gaze, 3) saccadic pursuit, and 4) markedly diminished caloric nystagmus. Gynecological examinations revealed recurrent ovarian cancer. Brain CT and brain MRI were normal. Cytological study of the cerebrospinal fluid was class I. Paraneoplastic brainstem encephalitis was diagnosed. Steroid pulse therapy improved her subjective symptoms and neuro-otological findings. We emphasize that the early induction of plasmapheresis or steroid pulse therapy should be used to treat patients with paraneoplastic neurologic syndrome and that neuro-oto-logical examinations are indispensable for quantitatively assessing the effectiveness of these treatments.
AbstractList We encountered two patients with paraneoplastic neurologic syndrome who visited our ENT clinic complaining of vertigo. The first patient was a 73-year-old woman who was admitted to our hospital because of dysequilibrium and dysarthria of subacute onset. Chest CT showed swelling of the mediastinal and right hilar lymphnodes. Pathological examination of the hilar lymphnodes taken by thoracotomy revealed small cell carcinoma of the lung. Brain CT and brain MRI were normal. Cytological study of the cerebrospinal fluid was class I. Paraneoplastic cerebellar degeneration was diagnosed. Neuro-otological examinations revealed: 1) gaze nystagmus on both right and left lateral gaze, 2) saccadic pursuit, 3) hypometric saccades, 4) diminished caloric nystagmus and 5) decreased visual suppression of caloric nystagmus. Treatment with plasmapheresis was successful to improve limb/truncal ataxia, dysarthria, and neuro-otological findings. The second patient was a 54-year-old woman who had had surgical treatment 3 years before for ovarian cancer. She visited our ENT clinic because of vigorous vertigo and diplopia of sudden onset. Neurological examinations revealed reduced deep tendon reflexes and neuro-otological examinations revealed: 1) bilateral abducens nerve palsy, 2) gaze nystagmus on both right and left lateral gaze, 3) saccadic pursuit, and 4) markedly diminished caloric nystagmus. Gynecological examinations revealed recurrent ovarian cancer. Brain CT and brain MRI were normal. Cytological study of the cerebrospinal fluid was class I. Paraneoplastic brainstem encephalitis was diagnosed. Steroid pulse therapy improved her subjective symptoms and neuro-otological findings. We emphasize that the early induction of plasmapheresis or steroid pulse therapy should be used to treat patients with paraneoplastic neurologic syndrome and that neuro-oto-logical examinations are indispensable for quantitatively assessing the effectiveness of these treatments.
Author Osanai, Ryuichi
Kinoshita, Masanobu
Tsubaki, Keiju
Namiki, Satoko
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  fullname: Namiki, Satoko
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References 12) Peterson K, Rosenblum MK, Kotanides H, et al: Paraneoplastic cerebellar degeneration: a clinical analysis of 55 anti-Yo antibodypositive patients. Neurology 42: 1931-1937, 1992
17) Moll JWB, Henzen-Longmans SC, van der Meche FGA, et al: Early diagnosis and in-travenous immune globulin therapy in para-neoplastic cerebellar degeneration [letter]. J Neurol Neurosurg Psychiatry 56: 112, 1993
19) Gulya AJ: Neurological paraneoplastic syndromes with neurotologic manifestations. Laryngoscope 103: 754-761, 1993
7) Bolla L, Palmer RM: Paraneoplastic cerebellar degeneration. Case report and literature review. Arch Intern Med 157: 1258-1262, 1997
6) Baloh RW: Paraneoplastic cerebellar disorders. Otolaryngol Head Neck Surg 112: 125-127,1995
11) 佐藤修三,犬塚貴:Paraneoplastic Syn-dromeのアンケート調査報告.厚生省特定疾患.免疫性神経疾患調査研究班.平成5年度研究報告書.26-28頁,1993
2) 廣瀬源二郎:傍悪性腫瘍症候群の診断基準・病型分類・重症度.内科75:1399-1402,1995
4) Dropcho EJ: Autoimmune CNS paraneoplastic disorders: Mechanisms, diagnosis and therapeutic options. Ann Neurol 37 Suppl 1: S102-S113, 1995
20) Posner JB: Written communication, 1991. cited in: Gulya AJ: Neurological paraneo-plastic syndromes with neurotologic manifestations. Laryngoscope 103: 754-761, 1993
3) Nath U, Grant R: Neurological paraneoplastic syndromes. J Clin Pathol 50: 975-980, 1997
1) 田中恵子:腫瘍随伴性小脳変性症.最新医50:484-489,1995
18) Stark E, Wurster U, Patzold U, et al: Immunological and clinical response to immunosuppressive treatment in paraneoplastic cerebellar degeneration. Arch Neurol 52
5) Dropcho EJ: Principles of paraneoplastic syndromes. Ann NY Acad Sci 841: 246-261, 1998
13) Anderson NE, Rosenblum MK, Graus F, et al: Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology 38: 1391-1398, 1988
14) Graus F, Abos J, Roquer J, et al: Effect of plasmapheresis on serum and CSF autoantibody levels in CSF paraneoplastic syndromes. Neurology 40: 1621-1623, 1990
16) Uchuya M, Graus F, Vega F, et al: Intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with antineuronal antibodies. J Neurol Neurosurg Psychiatry 60: 388-392, 1996
814-818,1995
15) Graus F, Vega F, Delattre JY, et al: Plasmapheresis and antineoplastic treatment in CNS paraneoplastic syndromes with antineuronal autoantibodies. Neurology 42: 536-540, 1992
8) 内藤理恵,室伏利久,水野正浩:悪性腫瘍の随伴性小脳変性症疑い例の神経耳科学的所見。耳鼻・頭頸外科69:906-909,1997
10) 田中正美,田中恵子:傍腫瘍性神経症候群.日内会誌87:670-677,1998
9) Nausieda PA, TannerCM, Weiner WJ: Opsoclonic cerebellopathq: Aparaneoplastic syndrome responsive to thiamine. Arch Neuro 138:780-781,1981
References_xml – reference: 18) Stark E, Wurster U, Patzold U, et al: Immunological and clinical response to immunosuppressive treatment in paraneoplastic cerebellar degeneration. Arch Neurol 52:
– reference: 814-818,1995
– reference: 19) Gulya AJ: Neurological paraneoplastic syndromes with neurotologic manifestations. Laryngoscope 103: 754-761, 1993
– reference: 6) Baloh RW: Paraneoplastic cerebellar disorders. Otolaryngol Head Neck Surg 112: 125-127,1995
– reference: 8) 内藤理恵,室伏利久,水野正浩:悪性腫瘍の随伴性小脳変性症疑い例の神経耳科学的所見。耳鼻・頭頸外科69:906-909,1997
– reference: 12) Peterson K, Rosenblum MK, Kotanides H, et al: Paraneoplastic cerebellar degeneration: a clinical analysis of 55 anti-Yo antibodypositive patients. Neurology 42: 1931-1937, 1992
– reference: 16) Uchuya M, Graus F, Vega F, et al: Intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with antineuronal antibodies. J Neurol Neurosurg Psychiatry 60: 388-392, 1996
– reference: 5) Dropcho EJ: Principles of paraneoplastic syndromes. Ann NY Acad Sci 841: 246-261, 1998
– reference: 10) 田中正美,田中恵子:傍腫瘍性神経症候群.日内会誌87:670-677,1998
– reference: 13) Anderson NE, Rosenblum MK, Graus F, et al: Autoantibodies in paraneoplastic syndromes associated with small-cell lung cancer. Neurology 38: 1391-1398, 1988
– reference: 9) Nausieda PA, TannerCM, Weiner WJ: Opsoclonic cerebellopathq: Aparaneoplastic syndrome responsive to thiamine. Arch Neuro 138:780-781,1981
– reference: 2) 廣瀬源二郎:傍悪性腫瘍症候群の診断基準・病型分類・重症度.内科75:1399-1402,1995
– reference: 4) Dropcho EJ: Autoimmune CNS paraneoplastic disorders: Mechanisms, diagnosis and therapeutic options. Ann Neurol 37 Suppl 1: S102-S113, 1995
– reference: 20) Posner JB: Written communication, 1991. cited in: Gulya AJ: Neurological paraneo-plastic syndromes with neurotologic manifestations. Laryngoscope 103: 754-761, 1993
– reference: 3) Nath U, Grant R: Neurological paraneoplastic syndromes. J Clin Pathol 50: 975-980, 1997
– reference: 15) Graus F, Vega F, Delattre JY, et al: Plasmapheresis and antineoplastic treatment in CNS paraneoplastic syndromes with antineuronal autoantibodies. Neurology 42: 536-540, 1992
– reference: 1) 田中恵子:腫瘍随伴性小脳変性症.最新医50:484-489,1995
– reference: 11) 佐藤修三,犬塚貴:Paraneoplastic Syn-dromeのアンケート調査報告.厚生省特定疾患.免疫性神経疾患調査研究班.平成5年度研究報告書.26-28頁,1993
– reference: 17) Moll JWB, Henzen-Longmans SC, van der Meche FGA, et al: Early diagnosis and in-travenous immune globulin therapy in para-neoplastic cerebellar degeneration [letter]. J Neurol Neurosurg Psychiatry 56: 112, 1993
– reference: 7) Bolla L, Palmer RM: Paraneoplastic cerebellar degeneration. Case report and literature review. Arch Intern Med 157: 1258-1262, 1997
– reference: 14) Graus F, Abos J, Roquer J, et al: Effect of plasmapheresis on serum and CSF autoantibody levels in CSF paraneoplastic syndromes. Neurology 40: 1621-1623, 1990
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Snippet We encountered two patients with paraneoplastic neurologic syndrome who visited our ENT clinic complaining of vertigo. The first patient was a 73-year-old...
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SubjectTerms brainstem encephalitis
ENG
eye movements
paraneoplastic neurologic Syndrome
Subacute cerebellar degeneration
Title Paraneoplastic Neurologic Syndrome: A Report of Two Patients
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