|LETTER TO THE EDITOR
|Ahead of print publication
Speech disorders in Fahr's disease: Hypokinetic, hyperkinetic, and mixed
Jamir P Rissardo, Ana L Fornari Caprara
Department of Medicine, Federal University of Santa Maria, Santa Maria, Brazil
|Date of Submission||11-Jan-2022|
|Date of Decision||22-Apr-2022|
|Date of Acceptance||22-Apr-2022|
Jamir P Rissardo,
Av. Roraima, 1000 - Camobi, Santa Maria, RS 97105-900
Source of Support: None, Conflict of Interest: None
We read the article entitled “A Rare Case of Fahr's Syndrome with Bilateral Vocal Cord Palsy” in the esteemed “Medical Journal of Dr. D.Y. Patil Vidyapeeth” with great interest. Goutham et al. reported a middle-aged female presenting with biphasic stridor. Video laryngoscopy showed both vocal cords in the paramedian position. A cranial computed tomography scan showed calcifications at the basal ganglia, dentate nucleus, and semioval centrum.
Herein, we would like to discuss speech abnormalities in Fahr's Disease (FD). Speech disorders are reported as a common symptom of bilateral striopallidodentate calcinosis. Also, it is noteworthy that slurred speech is considered a core clinical feature suggesting further investigation with neuroimaging. However, speech impairment is probably the most poorly described clinical manifestation of FD in the literature.
A literature search was performed in Medline using a set of terms that included “Fahr's Disease,” “speech,” and “voice without language restriction” [Supplementary Material – Other 1]. We found the following clinical descriptions for speech abnormalities: “slurred speech,” “speech disorder,” “speech difficulty,” “speech abnormalities,” “progressively slurred speech,” “slurring of speech,” “a deterioration of speech,” “occasional stuttering in her speech and slight hypophonia,” “occasional stuttering of speech in crowds,” “speech disturbance,” “mild dysarthric speech,” “speech impairment,” “speech problems,” and “speech deficit.”
We hypothesized three clinical speech disorders descriptions based on the basal ganglia physiological mechanism of language processing and motor function [Figure 1]. The speech abnormalities could be categorized into hypokinetic, hyperkinetic, and mixed (combined) disorders. The division is characterized by the main movement disorder found in the patient affected by FD such as Parkinsonism More Details, dystonia, and ataxia. A hypokinetic speech disorder is defined by monopitch, monoloudness, reduced loudness, and rapid rate characteristics. It is commonly encountered in individuals presenting with parkinsonism. On the other hand, hyperkinetic speech disorders present with inappropriate silences, voice stoppages, prolonged phonemes, and variable rates. Moreover, some individuals could have mixed features of hypokinetic and hyperkinetic speech disorders. These subjects may be classified as having a combined speech disorder.
|Figure 1: Speech Disorders in Fahr's Disease, also known as primary familial brain calcification and familial idiopathic basal ganglia calcification. Speech disorders can be classified into three categories: hypokinetic, hyperkinetic, and mixed (combined) disorders. The division is characterized by the main movement disorder found in the patient affected by FD|
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Santos et al. reported an elderly female with FD assessing stomatognathic and vocal changes. They observed mixed-(oral and nasal) breathing, pneumo-phono-articulatory incoordination, predominantly nasal resonance, reduction of maximum phonation time, monotonous voice quality with hoarse and trembling, nasality due to low mobility of the soft palate, slowness of speech, and the presence of tremors. Therefore, Santos et al. probably reported a patient with a hypokinetic speech disorder.
In 2020, Anil et al. described FD in a female child presenting with slow and sluggish oro-motor movements. She also had deficits in oro-motor functions with articulatory errors and reduced intelligibility of speech. Language assessment revealed reduced vocabulary and delay in expressive language. Thus, Anil et al. depicted a hyperkinetic movement disorder. It is noteworthy that the patient's age could have contributed to the diagnosis of the speech disorder.
The clinical distinction of speech abnormalities can help to provide a more detailed description and contribute to a better understanding of the cases. Also, it can promote further studies to investigate possible speech therapies such as Lee Silverman voice treatment.
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| References|| |
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Aydinli FE, Dumbak AB. The effects of Lee-Silverman voice treatment on voice and swallowing functions in a case with bilateral striopallidodentate calcinosis. Neurol India 2019;67:1522-4.
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Santos KW, Fraga BF, Cardoso MC. Dysfunctions of the stomatognathic system and vocal aspects in Fahr disease: Case report. Codas 2014;26:164-7.
Anil MA, Rebello RM, Bhat JS. Speech-language profile of a child with Fahr's disease: Case report of a rare neurodegenerative disorder. J Nat Sc Biol Med 2020;11:206-9. [Full text]