Annals of Movement Disorders

: 2022  |  Volume : 5  |  Issue : 3  |  Page : 194--197

Paraneoplastic dystonia and spastic ataxia syndrome as a manifestation of oral carcinoma: An evolving story

Saman Fatima1, Aanchal Kakkar2, Madhavi Tripathi3, Deepti Vibha1, Rajesh K Singh1, Manjari Tripathi1, Arunmozhimaran Elavarasi1,  
1 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Dr. Arunmozhimaran Elavarasi
Room no 706, Department of Neurology, All India Institute of Medical Sciences, New Delhi - 110 029


Paraneoplastic syndromes are a heterogeneous group of disorders that present with varied symptoms in the presence of a tumor but are unrelated to the growth of primary malignancies, invasiveness, or metastases. Paraneoplastic neurological syndromes are estimated to affect <1% of the patients with cancer. They are commonly associated with small-cell lung carcinoma and hematologic and gynecologic malignancies and are rarely observed in head and neck malignancies. The diagnosis of paraneoplastic neurological syndromes may precede, be concurrent with, or follow the diagnosis of a malignant tumor. In this report, we present the case of a 55-year-old man who presented with rapidly progressive spastic ataxia and neurobehavioral symptoms with a background of longstanding cervical dystonia, which subacutely progressed to generalized dystonia. The patient was diagnosed with oral squamous cell carcinoma.

How to cite this article:
Fatima S, Kakkar A, Tripathi M, Vibha D, Singh RK, Tripathi M, Elavarasi A. Paraneoplastic dystonia and spastic ataxia syndrome as a manifestation of oral carcinoma: An evolving story.Ann Mov Disord 2022;5:194-197

How to cite this URL:
Fatima S, Kakkar A, Tripathi M, Vibha D, Singh RK, Tripathi M, Elavarasi A. Paraneoplastic dystonia and spastic ataxia syndrome as a manifestation of oral carcinoma: An evolving story. Ann Mov Disord [serial online] 2022 [cited 2023 Jun 1 ];5:194-197
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Full Text


Paraneoplastic neurological syndromes (PNS) are rare immune-mediated neurological disorders that are triggered by tumors. They are treatable by addressing the underlying malignancies and neurological dysfunction. However, head and neck squamous cell cancers are rarely associated with PNS. To the best of our knowledge, our report presents the first case of PNS associated with oral carcinoma.

 Case Report

Our patient was a 55-year-old man who had cervical dystonia for 14 years and was in his usual state of health approximately 2.5 months before presentation. At presentation, he developed tremulousness of the upper limbs, decreased dexterity, and distal paresthesia. In addition, he developed imbalance while walking and swaying to either side, which progressed over the next 2 months to the extent that he could not walk or sit unsupported. He developed a slow and scanning speech and experienced behavioral disturbances. Furthermore, he experienced remarkable unintentional weight loss of 10 kg over 2 months. He had two seizures before the index admission, for which antiseizure medications were started. He was a reformed smoker (5 pack-years) and regularly consumed alcohol for 14 years.

On general examination, he was severely emaciated, and his body mass index was 16.93. There was a hard discrete lump at the floor of his mouth extending to the right hemimandible. He had generalized dystonia with left-sided laterocollis and dystonic neck tremor, oromandibular and truncal dystonia, and a wide-based gait. Higher mental function examination revealed inattention and predominant frontal lobe involvement with frontal release reflexes. His cranial nerves were intact. There was spasticity, weakness, and hyperreflexia in all four limbs with impaired finger–nose and heel–shin test (Video 1 [SUPPORTING:1]).

There was no obvious cerebellar atrophy on imaging (Figure 1]). Nerve conduction studies were normal. A biopsy of the mass from the floor of the mouth was performed, which showed moderately differentiated squamous cell carcinoma ([Figure 2]). The patient had paraneoplastic syndrome of inappropriate antidiuretic hormone secretion with resultant euvolemic hyponatremia. There was no hypercalcemia and paraneoplastic antibody panel (anti-Hu, Ri, Yo, Ma2, CV2/CRMP5, amphiphysin, Sox-1, Zic-4, GAD 65, anti-titin, anti-recoverin, and anti-Tr) of the cerebrospinal fluid was negative. The cerebrospinal fluid was acellular with elevated protein levels (80 mg/dl). The glucose level was 49 mg/dl (RBS 65 mg/dl), with unyielding work up for tuberculosis, syphilis, and bacterial and fungal infections. The patient’s antibody profile revealed antinuclear antigen positivity at 1:100 and nuclear membrane pattern; however, anti-ds DNA, antineutrophil cytoplasmic antibodies, rheumatoid factor, and extractable nuclear antigen profile were negative. Similarly, anti-N-methyl-d-aspartate, alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, contactin-associated protein-like 2, leucine-rich glioma-inactivated 1, and gamma aminobutyric acid-B receptor antibodies in serum were negative. Positron emission tomography–computed tomography showed a metabolically active lytic lesion in the right hemimandible with metastatic right cervical lymph nodes ([Figure 3]).{Figure 1} {Figure 2} {Figure 3}

The patient was treated with 1 gm of intravenous methylprednisolone for 5 days, with no significant clinical improvement. However, no palliative chemotherapy or radiotherapy options could be offered to him because of his worsening general and neurological conditions. The patient died 5 months after the diagnosis.


Paraneoplastic syndromes associated with head and neck cancer can be divided into six main groups: endocrine, dermatologic, hematologic, rheumatologic, neurologic, and ocular syndromes. As observed in our patient, paraneoplastic syndrome of inappropriate antidiuretic hormone secretion is most commonly associated with squamous cell carcinoma of the oral cavity.[1] Among PNS disorders associated with head and neck cancers, the most frequently reported syndromes have been cerebellar degeneration or Lambert–Eaton myasthenic syndrome.[2] Paraneoplastic cerebellar degeneration is suggested to be caused by antineuronal antibodies released by the tumor cells causing neuronal death.[3]

Among head and neck cancers, paraneoplastic cerebellar degeneration has been previously reported in patients with squamous cell carcinoma of the oropharynx, lymphoepithelial carcinoma of the tonsils and spindle cell carcinoma of the nasal cavity.[4],[5],[6] All three patients presented with insidious onset ataxia and slurring of speech. To the best of our knowledge, ours is the first case to report symptoms of dystonia, spastic quadriparesis, and sensory neuropathy in addition to cerebellar symptoms. Classical PNS has not yet been reported to be associated with squamous cell carcinoma of the mandible.

According to the updated criteria for PNS, three levels of certainty have been created, according to the coherence between clinical phenotype, antibody, and cancer. However, these criteria come with the caveat of underestimating the occurrence of PNS without onconeural antibodies as they lay importance on antibody positivity.

Our patient was diagnosed with definite paraneoplastic encephalitis, according to the 2004 PNS criteria, which classified three levels of evidence for PNS: definite, probable, and possible.[7] In addition, based on the recently updated PNS–Care score, he reached the diagnostic certainty level of possible PNS.[8]


The treatment for PNS is directed at the underlying malignancies. However, the early destruction of neural structures prevents clinical improvement in some patients, despite treatment of the underlying lesions.

Multiaxial involvement of the neuraxis in the form of mixed hyperkinetic movement disorders has a wide range of differentials. Rapid progression and constitutional symptoms should encourage the search for malignancy. Although rare, squamous cell carcinoma of the oral cavity may lead to PNS.


We thank the patient and the family for their support and consent to publish the case for educational value.

Author contribution

Saman Fatima was involved in the conceptualization of the report, diagnosis and management of the patient and in writing the first draft of the manuscript

Arunmozhimaran Elavarasi was involved in the conceptualization of the report, diagnosis and patient care, and in review and critique of the manuscript

Aanchal Kakkar was involved in the histopathologic diagnosis and review and critique of the manuscript

Madhavi Tripathi was involved in the interpretation of the PET scan and review and critique of the manuscript

Manjari Tripathi, Deepti Vibha and Rajesh Kumar Singh were involved in the diagnosis and management of the case and in critique and review of the manuscript.

Ethical compliance statement

Patient consent taken. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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