Correspondence Address: Dr. Vishnupriya Veeraraghavan Department of Psychiatry, Aarupadaiveedu Medical College and Hospital, Puducherry India
Source of Support: None, Conflict of Interest: None
Dystonia is a movement disorder whose main feature is a sustained or intermittent muscle contraction causing abnormal, often repetitive, movements. Cervical dystonia (CD) is a type of focal dystonia affecting cervical muscles leading to abnormal postures and movements of the head, neck, and shoulders. Pathological laughter and crying are a condition characterized by uncontrollable episodes of laughter and crying. It occurs without any apparent triggering stimulus or in response to a stimulus which had not resulted in cry or laughter before the onset of the condition. It is characterized as a disorder of emotional expression rather than a disorder of feelings. The purpose of presenting this case report is that this patient presented with pathological laughter and CD without any other neurological complications which are a very rare and unusual presentation.
How to cite this article: Veeraraghavan V, Srinivasan K, Alexander JD. A rare co occurrence of Cervical Dystonia and Pathological Laughter in an elderly female. J Geriatr Ment Health 2020;7:113-5
How to cite this URL: Veeraraghavan V, Srinivasan K, Alexander JD. A rare co occurrence of Cervical Dystonia and Pathological Laughter in an elderly female. J Geriatr Ment Health [serial online] 2020 [cited 2021 Jun 25];7:113-5. Available from: https://www.jgmh.org/text.asp?2020/7/2/113/307588
Dystonia is a movement disorder whose main feature is a sustained or intermittent muscle contraction causing abnormal, often repetitive, movements. Cervical dystonia (CD) is a type of focal dystonia affecting cervical muscles leading to abnormal postures and movements of the head, neck, and shoulders., Dystonic movements are twisting, and tremulous. It is characterized as a disorder of emotional expression rather than a disorder of feelings. The lifetime risk for patients with CD meeting diagnostic criteria for depression is 15' for anxiety disorders is 26.4'. The prevalence of pathological laughter and crying in individual diagnostic categories was: 4.7' (18/387) of idiopathic Parkinson's disease (PD), 2.7' (2/74) of primary dystonia, 3.1' (2/65) of essential tremor, and 7.8' (8/108) of patients with other forms of Parkinsonism More Details. Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation. The term emotional lability (EL) was first described by Pierre-Marie in 1892. The term pathological crying and laughing (PCL) was used by Wilson in the year 1924, in which he categorized his unifying theory of the disorder “PCL,” is used interchangeably with “pseudobulbar affect,” “EL,” and “involuntary emotional expression disorder.” It can be differentiated from mood disorders in which laughter or crying is associated with periods of happiness or sadness and from regular laughter and crying which is concurrent with the triggering stimulus. Pathological laughter and crying are associated with common in several neurological conditions including, in order of prevalence: motor neuron disease (MND), traumatic brain injury, multiple sclerosis (MS), stroke, multiple system atrophy (MSA)-cerebellar type, Alzheimer's disease, and PD.,,,,, Pathological laughter in a case of CD without any degenerating features in the brain is a relatively rare occurrence, and hence we report a case.
A 70-year-old female presents to the psychiatry outpatient department with complaints of dull aching pain in the nape of the neck and shoulders for 3 years with the movement of the head toward the left. She was from a rural area and her occupation is carrying wooden logs on her neck for long distances. Involuntary uncontrollable low pitch laughter for the past 8 months. She assumes a sensory trick for getting relieved from the pain and abnormal head pulling by touching her right cheek with her arm. Involuntary sudden outbursts of laughter increased in frequency in the past 3 months. It was elicited by nonspecific, trivial, and neutral stimuli (such as enquiry about her health) that lasted for a few minutes until she gained some control. It was not associated with any emotionally provoking situations. Her personal and social behavior was entirely appropriate. She did not have any comorbid medical illness Video 1, no family history of psychiatric or neurological illness, and no history of any psychiatric illness. She was not on any medications previously and did not undergo any prior treatment for her above-mentioned complaints.
On examination, left-sided sternocleidomastoid is taut and she moves her chin downward frequently and supports her right cheek with her right arm to mitigate the pain (Geste antagoniste). Systemic examination findings revealed 60° head rotation to the left and left shoulder elevation and a taut sternocleidomastoid. The Toronto Western Spasmodic Torticollis Rating Scale score was 42. The fundus examination was normal. The following psychological assessment scales were applied. University of Florida Modified PBA Screening Question test was positive. Central nervous system-Lability Scale (CNS-LS) score was 13. Mini-Mental State Examination score was 20. Beck depression inventory and the Beck Anxiety Inventory score dint showed significant scores for depression and anxiety. Baseline investigations done were normal. Vitamin B12 levels were normal and thyroid function test revealed a normal T3 and T4 levels, but thyroid-stimulating hormone was 8.8 ng/ml. Her metabolic profile was normal. Electroencephalogram done during laughter was normal. Computed tomography of the brain showed ill-defined hypodense areas seen involving bilateral periventricular white matter suggestive of small-vessel disease and magnetic resonance imaging (MRI) of the brain confirmed small-vessel disease. She was started on selective serotonin reuptake inhibitor tablet sertraline 50 mg, tablet clonazepam 0.5 mg, and tablet baclofen 20 mg. Neurologist opinion was sort and advised tablet trihexyphenidyl 6 mg divided doses, tablet aspirin/atorvastatin 75/10 mg, and botulinum toxin in the subsequent visits if dystonic movements persisted. After seeking neurologist opinion [Figure 1], both tablet baclofen and clonazepam were tapered and stopped. After 1 week, the frequency of her laughter episodes reduced. CNS-LS scores were 8. Neurologist review was again sorted before discharge and tablet tihexiphenidyl dose was tapered to 4 mg. She was discharged with tablet sertraline 50 mg and tablet trihexyphenidyl 4 mg. After 1 month of discharge, she came for follow-up, laughter episodes were there only occasionally and pain in the neck reduced, dystonic movements reduced in intensity.
Figure 1: Image of the patient with cervical dystonia
CD also known as spasmodic torticollis is the most common type of focal dystonia. It usually begins between the ages of 30 and 50 years, often with initial neck stiffness and restricted head mobility. The neck and shoulder pain occurs in 75' of cases. Sensory tricks (Geste antagoniste) such as lightly touching the face or chin reduce the severity of symptoms in most patients. The differential diagnosis includes essential head tremor, tardive dystonia in which retrocollis is common, anterocollis caused by cervical myopathy or MSA, and secondary torticollis associated with a neck injury, atlantoaxial dislocation, cervical disk disease, spinal cord neoplasm, or soft-tissue infections of the neck. Our case report is unique, as fixed dystonia with pathological laughter has few reports till date. On examination, there was no focal neurological sign. It was a focal, static, isolated with onset at late adulthood. There was no history of psychiatric treatment. Overuse CD should be suspected when typical symptoms and signs of CD develop in the context of chronic repetitive use or overuse of cervical muscles, especially where exacerbating tasks involve asymmetric postures. CD has rarely been associated with chronic overuse, with very few case reports. The absence of neurodegenerative changes was again a fascinating point in this patient as the pseudobulbar effect is commonly associated with neurodegenerative disorders such as MND or MS. Anatomical correlates between CD and pathological laughter is the involvement of basal ganglia and cerebellum. The cerebellum plays a vital role in modulating emotional responses and keep them appropriate to the social situation and the patient's mood based on input from the cerebral cortex. Disruption of corticopontine–cerebellar circuits results in impairment of this cerebellar modulation, causing pathological laughter. Motor cortex including the frontal and temporal cortices sends inputs to the brainstem which is modulated by the cerebellum. The motor input is mediated by inhibitory input from the somatosensory cortex. Reduced inhibitory input results in disinhibition of the cerebellum, leading to pathological laughter. Studies prove that patients with CD had an abnormal cerebellar cortical connectivity at rest and the plasticity of this cerebellar pathway is also altered. Even in the absence of overt cerebellar signs, cerebellar dysfunction was identified in most patients with CD. Although we are unable to establish any basal ganglia or cerebellum function in our patient through MRI, still higher imaging techniques such as functional MRI can be done to establish a neurobiological correlate.
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The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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