“Dancing Eye Syndrome” Secondary to Opsoclonus-Myoclonus Syndrome in Small-Cell Lung Cancer

Abstract

Among paraneoplastic neurologic disorders (PND), opsoclonus-myoclonus syndrome, so-called “dancing eye syndrome,” is a rare disorder combining multivectorial eye movements, involuntary multifocal myoclonus, and cerebellar ataxia. Although several paraneoplastic antibodies against postsynaptic or cell-surface antigens have been reported, usually most patients are serum antibody negative. We report a 65-year-old patient with opsoclonus-myoclonus syndrome revealing a small-cell lung carcinoma. If serologic antineuronal anti-body screening was negative, autoantibodies against glutamic acid decarboxylase (anti-GAD) were positive. Despite the specific anticancer treatment and high dose corticosteroids, the patient developed a severe and progressive encephalopathy and died 10 days later.

Paraneoplastic opsoclonus-myoclonus-ataxia syndrome revealing dual malignancy

“…Antineuronal antibodies have been occasionally detected in OMA. Among them, Anti-Neuronal Nuclear Antibody2 (ANNA-2 also called anti-RI) is the most frequently associated with the dancing eye syndrome [8]. Although these antibodies have been also rarely detected in SCLC [5], they were negative in our patient.…”

“…Only bronchial biopsies with immunohistochemistry helped to confirm the histologically different nature of the second tumour concluding to concurrent SCLC. Paraneoplastic OMS has been previously reported in SCLC, ovarian and breast malignancies in adults [8]. Some researchers suggest an immune-mediated dysfunction of premotor neurons of the brainstem or disinhibition of cerebellar nuclei [5].…”

Paraneoplastic opsoclonus-myoclonus-ataxia syndrome revealing dual malignancy

“…Antineuronal antibodies have been occasionally detected in OMA. Among them, Anti-Neuronal Nuclear Antibody2 (ANNA-2 also called anti-RI) is the most frequently associated with the dancing eye syndrome [8]. Although these antibodies have been also rarely detected in SCLC [5], they were negative in our patient.…”

“…Only bronchial biopsies with immunohistochemistry helped to confirm the histologically different nature of the second tumour concluding to concurrent SCLC. Paraneoplastic OMS has been previously reported in SCLC, ovarian and breast malignancies in adults [8]. Some researchers suggest an immune-mediated dysfunction of premotor neurons of the brainstem or disinhibition of cerebellar nuclei [5].…”

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

“…One had West Nile encephalitis with positive anti-GAD antibodies; another had a “paraneoplastic centrally mediated disorder with central planning” but had no detectable antibody; and the remaining case had isolated opsoclonus–myoclonus with serum anti-GAD antibodies. 30 , 31 Ten cases documented the time between symptom onset and presentation with a median of 3 months and ranging from weeks up to 13 years. Fifteen patients described gait difficulties, 11 reported pain, 12 had cramps or spasms, and 13 had limb stiffness.…”

Treatment-refractory paraneoplastic opsoclonus–myoclonus syndrome in a patient with small-cell carcinoma of the lung

“…In addition, there are also limited number of OMS cases in literature. [9][10][11]14 While in some cases, the OMS was the initial presentation of the malignancy, some of the cases were initial clues for progression. Similar with the experience by Nadal et al, 9 we did not show any radiological progression, only clinical progression by paraneoplastic syndrome and biochemical progression by increase in CEA were detected.…”

“…However, in some of them, the disease was refractory. 14,16 In our case, we tried all the maneuvers that have been claimed to be effective in paraneoplastic OMS. In addition, clonazepam and topiramate were also ineffective.…”

Paraneoplastic opsoclonus-myoclonus-ataxia syndrome revealing dual malignancy

“…Antineuronal antibodies have been occasionally detected in OMA. Among them, Anti-Neuronal Nuclear Antibody2 (ANNA-2 also called anti-RI) is the most frequently associated with the dancing eye syndrome [8]. Although these antibodies have been also rarely detected in SCLC [5], they were negative in our patient.…”

“…Only bronchial biopsies with immunohistochemistry helped to confirm the histologically different nature of the second tumour concluding to concurrent SCLC. Paraneoplastic OMS has been previously reported in SCLC, ovarian and breast malignancies in adults [8]. Some researchers suggest an immune-mediated dysfunction of premotor neurons of the brainstem or disinhibition of cerebellar nuclei [5].…”

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

“…One had West Nile encephalitis with positive anti-GAD antibodies; another had a “paraneoplastic centrally mediated disorder with central planning” but had no detectable antibody; and the remaining case had isolated opsoclonus–myoclonus with serum anti-GAD antibodies. 30 , 31 Ten cases documented the time between symptom onset and presentation with a median of 3 months and ranging from weeks up to 13 years. Fifteen patients described gait difficulties, 11 reported pain, 12 had cramps or spasms, and 13 had limb stiffness.…”

Treatment-refractory paraneoplastic opsoclonus–myoclonus syndrome in a patient with small-cell carcinoma of the lung

“…In addition, there are also limited number of OMS cases in literature. [9][10][11]14 While in some cases, the OMS was the initial presentation of the malignancy, some of the cases were initial clues for progression. Similar with the experience by Nadal et al, 9 we did not show any radiological progression, only clinical progression by paraneoplastic syndrome and biochemical progression by increase in CEA were detected.…”

“…However, in some of them, the disease was refractory. 14,16 In our case, we tried all the maneuvers that have been claimed to be effective in paraneoplastic OMS. In addition, clonazepam and topiramate were also ineffective.…”

Paraneoplastic opsoclonus-myoclonus-ataxia syndrome revealing dual malignancy

“…Antineuronal antibodies have been occasionally detected in OMA. Among them, Anti-Neuronal Nuclear Antibody2 (ANNA-2 also called anti-RI) is the most frequently associated with the dancing eye syndrome [8]. Although these antibodies have been also rarely detected in SCLC [5], they were negative in our patient.…”

“…Only bronchial biopsies with immunohistochemistry helped to confirm the histologically different nature of the second tumour concluding to concurrent SCLC. Paraneoplastic OMS has been previously reported in SCLC, ovarian and breast malignancies in adults [8]. Some researchers suggest an immune-mediated dysfunction of premotor neurons of the brainstem or disinhibition of cerebellar nuclei [5].…”

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

“…One had West Nile encephalitis with positive anti-GAD antibodies; another had a “paraneoplastic centrally mediated disorder with central planning” but had no detectable antibody; and the remaining case had isolated opsoclonus–myoclonus with serum anti-GAD antibodies. 30 , 31 Ten cases documented the time between symptom onset and presentation with a median of 3 months and ranging from weeks up to 13 years. Fifteen patients described gait difficulties, 11 reported pain, 12 had cramps or spasms, and 13 had limb stiffness.…”

Treatment-refractory paraneoplastic opsoclonus–myoclonus syndrome in a patient with small-cell carcinoma of the lung

“…In addition, there are also limited number of OMS cases in literature. [9][10][11]14 While in some cases, the OMS was the initial presentation of the malignancy, some of the cases were initial clues for progression. Similar with the experience by Nadal et al, 9 we did not show any radiological progression, only clinical progression by paraneoplastic syndrome and biochemical progression by increase in CEA were detected.…”

“…However, in some of them, the disease was refractory. 14,16 In our case, we tried all the maneuvers that have been claimed to be effective in paraneoplastic OMS. In addition, clonazepam and topiramate were also ineffective.…”