The Challenge
FigureTongue protrusion in patient. A. Severe tongue protrusion dystonia. B. Tongue protrusion ceased when a grape was inserted in the mouth.
Can you make the diagnosis?
The diagnosis:
Parkinsonism Relat Disord. 2019; 59: 146-150
and is characterized by repetitive and stereotypic movements of the face, mouth, and tongue, often with trunk and extremity involvement.
Parkinsonism Relat Disord. 2019; 59: 146-150
The mean prevalence rate of tardive dyskinesia in psychiatric patients who take antipsychotics is approximately 25%, although this is substantially higher with first-generation (for example, haloperidol) than with second-generation antipsychotics (for example, risperidone).
Parkinsonism Relat Disord. 2019; 59: 151-154
The symptoms persist after the drug is withdrawn.
Parkinsonism Relat Disord. 2019; 59: 151-154
The “lip-smacking” and tongue protrusion symptoms can be stopped with sensory input (for example, when a finger or object touches the lips).
Parkinsonism Relat Disord. 2019; 59: 146-150
Parkinsonism Relat Disord. 2019; 59: 146-150
) and did not improve with the administration of tetrabenazine and clonazepam. Her family declined other proposed interventions such as botulinum toxin injections into the genioglossus and bilateral pallidal deep brain stimulation.
- Schneider S.A.
- Aggarwal A.
- Bhatt M.
- et al.
Neurology. 2006; 67: 940-943
Differential Diagnosis
- Schneider S.A.
- Aggarwal A.
- Bhatt M.
- et al.
Neurology. 2006; 67: 940-943
,
Arch Gen Psychiatry. 1982; 39: 486-487
Tongue protrusion movements in edentulism-related oral dyskinesia are less striking than those of tardive dyskinesia and stop when opening the mouth; these patients can keep their tongue protruded voluntarily for several seconds, and there are no vermicular movements of the tongue when within the mouth.
Ann Neurol. 1983; 13: 97-99
Accordingly, the differential diagnosis for this patient should focus on neurodegenerative conditions.
Neuroacanthocytosis
- Dulski J.
- Sołtan W.
- Schinwelski M.
- et al.
Clin Neurol Neurosurg. 2016; 147: 78-83
This group mainly comprises autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome. Orofacial dyskinesia develops in most patients with neuroacanthocytosis.
- Dulski J.
- Sołtan W.
- Schinwelski M.
- et al.
Clin Neurol Neurosurg. 2016; 147: 78-83
Diagnosis is confirmed via genetic testing and chorein expression detection,
- Peikert K.
- Danek A.
- Hermann A.
Eur J Med Genet. 2018; 61: 699-705
although the initial detection often relies on blood smears.
- Storch A.
- Kornhass M.
- Schwarz J.
J Neurol. 2005; 252: 84-90
Pantothenate kinase-associated neurodegeneration
- Di Meo I.
- Carecchio M.
- Tiranti V.
J Inherit Metab Dis. 2019; 42: 49-56
Clinical manifestation includes motor disorder, cognitive and visual impairment, and, occasionally, psychiatric issues. Dystonia tends to affect the tongue, causing dysarthric speech and impaired swallowing and feeding.
- Saeedi Y.
- Kazemi F.
- Habibi S.A.H.
- et al.
Pediatr Neurol. 2020; 103: 76-78
The hallmark of this disease is a hyperintense lesion surrounded by hypointensity in the globus pallidus on MRI (eye of the tiger sign).
- Razmeh S.
- Habibi A.H.
- Orooji M.
- Alizadeh E.
- Moradiankokhdan K.
- Razmeh B.
Neurol Int. 2018; 10: 7516
Wilson disease
- Mulligan C.
- Bronstein J.M.
Neurol Clin. 2020; 38: 417-432
Clinical manifestations include liver disease and neuropsychiatric symptomatology. The most common neurologic symptom is dysarthria, followed by cerebellar ataxia, dystonia, parkinsonism, postural tremor, and dysphagia.
- Mulligan C.
- Bronstein J.M.
Neurol Clin. 2020; 38: 417-432
In a series that included 136 patients diagnosed with Wilson disease with neurologic signs, tongue involvement (lingual dystonia) was the initial symptom in 17% of juveniles and 13% of adults.
J Neurol Neurosurg Psychiatry. 1992; 55: 692-696
Several laboratory investigations are helpful in establishing the diagnosis, including serum ceruloplasmin, serum copper, and 24-hour urinary copper levels.
- Mulligan C.
- Bronstein J.M.
Neurol Clin. 2020; 38: 417-432
Neuroferritinopathy
in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews. University of Washington,
2005
Although dystonia and dysarthria are the main manifestations, neuroferritinopathy can also manifest as tremor, cerebellar ataxia, parkinsonism, dystonia, chorea, and cognitive decline.
in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews. University of Washington,
2005
Most patients develop a characteristic speech-related orofacial dystonia (dysarthrophonia) and orolingual dyskinesia.
in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews. University of Washington,
2005
The diagnosis can be reached by means of combining low serum ferritin measurements, MRI, and genetic testing.
- Tarnacka B.
- Jopowicz A.
- Maślińska M.
Int J Mol Sci. 2021; 22: 7820
Conclusions
Tongue dyskinesia is a movement disorder that can cause serious impairment of swallowing, eating, and breathing. Dentists should be aware that tongue dyskinesia is usually secondary to drugs but can also be a sign of an underlying neurodegenerative medical condition.
Supplemental Data
References
Setting the record straight: the nosology of tardive syndromes.
Parkinsonism Relat Disord. 2019; 59: 146-150
Tardive dyskinesia: who gets it and why.
Parkinsonism Relat Disord. 2019; 59: 151-154
Severe tongue protrusion dystonia: clinical syndromes and possible treatment.
Neurology. 2006; 67: 940-943
Research diagnoses for tardive dyskinesia.
Arch Gen Psychiatry. 1982; 39: 486-487
Edentulous orodyskinesia.
Ann Neurol. 1983; 13: 97-99
Clinical variability of neuroacanthocytosis syndromes: a series of six patients with long follow-up.
Clin Neurol Neurosurg. 2016; 147: 78-83
Current state of knowledge in chorea-acanthocytosis as core neuroacanthocytosis syndrome.
Eur J Med Genet. 2018; 61: 699-705
Testing for acanthocytosis: a prospective reader-blinded study in movement disorder patients.
J Neurol. 2005; 252: 84-90
Inborn errors of coenzyme A metabolism and neurodegeneration.
J Inherit Metab Dis. 2019; 42: 49-56
Tongue protrusion dystonia in pantothenate kinase-associated neurodegeneration.
Pediatr Neurol. 2020; 103: 76-78
Pantothenate kinase-associated neurodegeneration: clinical aspects, diagnosis and treatments.
Neurol Int. 2018; 10: 7516
Wilson disease: an overview and approach to management.
Neurol Clin. 2020; 38: 417-432
Wilson’s disease: the problem of delayed diagnosis.
J Neurol Neurosurg Psychiatry. 1992; 55: 692-696
Neuroferritinopathy.
in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews. University of Washington,
2005Copper, iron, and manganese toxicity in neuropsychiatric conditions.
Int J Mol Sci. 2021; 22: 7820
Biography
Dr. Diniz Freitas is an adjunct professor of Special Care Dentistry, Medical-Surgical Dentistry Research Group, Health Research Institute of Santiago de Compostela, University of Santiago de Compostela, Santiago de Compostela, Spain.
Biography
Dr. Diz Dios is a professor of Special Care Dentistry, Medical-Surgical Dentistry Research Group, Health Research Institute of Santiago de Compostela, University of Santiago de Compostela, Santiago de Compostela, Spain.
Biography
Dr. Fernández Feijoo is an associate professor of Special Care Dentistry, Medical-Surgical Dentistry Research Group, Health Research Institute of Santiago de Compostela, University of Santiago de Compostela, Santiago de Compostela, Spain.
Biography
Dr. Limeres Posse is an associate professor of Special Care Dentistry, Medical-Surgical Dentistry Research Group, Health Research Institute of Santiago de Compostela, University of Santiago de Compostela, Santiago de Compostela, Spain.
Biography
Dr. Outumuro Rial is an adjunct professor of Special Care Dentistry, Medical-Surgical Dentistry Research Group, Health Research Institute of Santiago de Compostela, University of Santiago de Compostela, Santiago de Compostela, Spain.
Article Info
Publication History
Published online: May 13, 2022
Publication stage
In Press Corrected Proof
Footnotes
Disclosures. None of the authors reported any disclosures.
Identification
DOI: https://doi.org/10.1016/j.adaj.2022.03.010
Copyright
© 2022 American Dental Association. All rights reserved.
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