Atypical parkinsonism and self-mutilation: A new lens on the old concept.

We report a case of atypical parkinsonism and self-mutilation.

INTRODUCTION

Atypical parkinsonism defines a syndrome that consists of parkinsonian features, such as akinesia, rigidity, and tremor, and additional clinical signs which are atypical for Parkinson's disease. These signs include early cognitive decline, severe dysautonomia, and early fall, but self‐mutilation has not been reported in atypical parkinsonism. Herein, we report a case of atypical parkinsonism and self‐mutilation.

CASE REPORT

A 69‐year‐old and high educated woman was referred to our Movement Disorders Clinic because of vertigo and bradykinesia in the last 4 months. She complained of right‐hand tremor for 3 months, several episodes of fall, insomnia, memory decline, depression, and urinary retention and frequency during the last months. Also, she reported self‐mutilation behaviors for 2 years such as scratching her skin around the umbilical area, which not accompanied by itching, and she felt relaxed after looking at her blood. (Figure 1) She denied any history of hallucination, obsessive‐compulsive behaviors, and other psychiatric disorders.

FIGURE 1

Self‐inflicted wounds of the abdomen on the periumbilical area

She has a history of diabetes mellitus, hypothyroidism, hypertension, bariatric surgery 7 years ago, and cholecystectomy 2 years ago. She was taking Metformin, Insulin, Metoprolol, Levothyroxine, and Fluoxetine. Her family history was unremarkable.

On clinical examination, she had bradykinesia, bilateral jerky tremor on hands during rest and postural holding, rigidity, wide‐based gait, stooped posture, pizza sign, and positive pull test; also, her MOCA was 22. Furthermore, her distal extremities were cold and pale, and her systolic and diastolic blood pressure fell 30 mm/Hg and 20 mm/Hg, respectively, after 3 minutes standing. Brain MRI showed non‐specific white matter lesions, but no cerebellar or pontine atrophy at that time. She did not have dystonia, chorea, and pyramidal signs.

We started the following regimen: Levodopa/Benzenoid 100/25 mg gradually increased to four times a day and Donepezil 2.5 mg a day. On follow‐up, the severity of self‐mutilation decreased but was not completely resolved, and her parkinsonism dramatically improved, also, she had not experienced falling again, but she increased levodopa/Benzenoid herself to 1.5 tablets four times a day, which caused reemerge of unsteadiness and falling and worsening of self‐mutilation, and reducing Levodopa/Benzenoid to the previous dosage improved falling but not self‐mutilation. On the one‐year follow‐up, she remained stable on the current regimen in addition to rehabilitation due to wide‐based gait and unsteadiness, but still has self‐mutilation, which is not bothering her.

DISCUSSION

The well‐known differential diagnosis of parkinsonism and self‐mutilation is Lesch‐Nyhan Syndrome (LNS), which has a central deficiency of dopamine similar to parkinsonism, the age at which dopaminergic neurons are disturbed can explain the different symptoms detected in these two conditions. , Upregulation of the striatal D2 receptor occurs in LNS, which is comparable to what can be seen in Parkinson's disease (PD). Therefore, the pathophysiology of hyperkinetic involuntary movements of LNS might be alike to levodopa‐induced dyskinesia. Moreover, there are reports that showed bilateral stimulation of the globus pallidus internus (GPi) can improve self‐mutilation in LNS, which points to the fact that self‐injurious behavior may be associated with basal ganglia dysfunction.

Self‐injurious behavior mostly seen in children and adolescents and infrequently initiates in adulthood. Self‐injurious behavior is mainly related to tic severity, obsessive‐compulsive disorder, and attention‐deficit/hyperactivity disorder, which further support the role of dopamine in the pathophysiology of self‐mutilation behaviors.

Two cases with SCA17 have been reported, both of them had chorea, ataxia, and cognitive decline. Although the patients did not have parkinsonism, parkinsonism can a manifestation of SCA17.

Consequently, self‐mutilation theoretically possible in parkinsonism, and to the best of our knowledge, this is the first report of self‐mutilation in a patient with atypical parkinsonism, that would lead to a better understanding of the underlying pathophysiological mechanism.

AUTHOR CONTRIBUTIONS

MS: Conception, Organization, Execution, Review and Critique. ME: Execution, Review and Critique. KG: Writing of the first draft.

ETHICAL APPROVAL

We hereby confirm that the present study conforms to the ethical standards and guidelines of the journal.