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Year : 2017  |  Volume : 65  |  Issue : 5  |  Page : 1144--1145

Anarthria in a patient with bilateral striopallidodentate calcinosis

Shoba Sreenath Meera1, N Shivashankar1, Ravi Yadav2, Maya Dattatraya Bhat3,  
1 Department of Speech Pathology and Audiology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore, Karnataka, India
2 Department of Neurology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore, Karnataka, India
3 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore, Karnataka, India

Correspondence Address:
Shoba Sreenath Meera
Room No. 19, G-14, OPD Block, Department of Speech Pathology and Audiology, NIMHANS, Hosur Road, Bangalore - 560 029, Karnataka

How to cite this article:
Meera SS, Shivashankar N, Yadav R, Bhat MD. Anarthria in a patient with bilateral striopallidodentate calcinosis.Neurol India 2017;65:1144-1145

How to cite this URL:
Meera SS, Shivashankar N, Yadav R, Bhat MD. Anarthria in a patient with bilateral striopallidodentate calcinosis. Neurol India [serial online] 2017 [cited 2021 Oct 28 ];65:1144-1145
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Bilateral striopallidodentate calcinosis (BSPDC) is a rare, autosomal dominant, inherited, degenerative, neurological condition which is characterised by symmetrical intracranial calcifications usually in the basal ganglia and dentate nuclei.[1] BSPDC has been erroneously also called as Fahr's disease.[1] However, the use of the term ‘Fahr's disease’ for this entity has been criticised and the use of the term ‘BSPDC’ is preferred.[2] Most cases with BSPDC present with movement disorders. Amongst these, Parkinsonism is reported to be present in over half of the cases, while hyperkinetic movement disorders (chorea, tremor, dystonia, athetosis and oro-facial dyskinesia) account for the rest. Other clinical manifestations include cognitive decline, cerebellar signs, dysarthria, and in a small proportion of cases, psychiatric features, pyramidal signs and gait disturbances.[1],[2]

Basal ganglia lesions produce dysarthria of varying degrees. Dysarthria in its most severe form is often referred to as anarthria – a complete loss of speech due to severe loss of neuromuscular control over speech with bilateral involvement of neural substrates that control speech production.[3] Language and cognitive abilities of patients with anarthria may be intact.[3]

A 39-year old, right-handed, literate, male, belonging to a middle socioeconomic stratum and a teacher by profession, presented with complaints of gradual worsening of balance since four years, progressive walking difficulty since three years, and extreme difficulty in speaking since one-and-a-half years. He was born of a second-degree consanguineous parentage. At the time of evaluation, the patient was completely dependent for all activities of daily living and had no speech production except for occasional production of undifferentiated sounds.

All the biochemical parameters including serum calcium, serum phosphorous, serum alkaline phosphatase, parathyroid hormone levels, vitamin D levels, renal function tests, liver function tests, hemogram, and routine urine examination were normal. The CT scan [Figure 1] revealed extensive calcification of bilateral cerebellar hemispheres, dentate nuclei, thalami, substantia nigra, basal ganglia, and centrum semiovale. Patchy calcifications were also noted in the cerebral cortex and subcortical white matter. Detailed neurological, bio-chemical and radiological evaluation with a focus on differential diagnosis between BSPDC and endocrine causes (e.g. hypoparathyroidism), implicated the diagnosis of BSPDC. The patient was referred to speech-language pathologists for evaluation of speech difficulties and to institute intervention strategies, which is the primary focus of this paper.{Figure 1}

A detailed oral-vocal mechanism examination revealed minimal jaw opening, poor speech and range of motion of the lips and palate, restricted sluggish and spastic tongue movements, no facial weakness, absence of volitional cough, and persistent drooling with salivary pooling in the oral cavity. The maximum phonation duration and diadochokinesis could not be assessed due to the inability to produce syllables. The patient did not report any difficulty in swallowing. However, due to restricted jaw and tongue movements, the patient could not form a bolus of food and hence, was on a semi-solid diet. The Frenchay Dysarthria Assessment[4] was administered and the scores revealed near-normal function only in the reflexes section (score of ‘a'/'b'), and almost no function (i.e., score of ‘d'/'e') in the other sections namely, the respiratory function, the lips, jaw, palate, laryngeal and tongue functions. On the auditory verbal comprehension (AVC) section of the Western Aphasia Battery,[5] the patient scored 200/200, suggesting no deficits in the AVC score. Further, the patient was screened based on a list of indigenously developed cognitive linguistic tasks and it was found that the patient was able to appreciate humour, pick the right interpretation of a proverb, sequence events and identify semantic and syntactic anomalies - all through nonverbal modes (by pointing as well as making a few facial expressions). A diagnosis of essentially normal cognitive linguistic abilities and presence of anarthria secondary to BSPDC was made.

Since the patient was doing well cognitively, he resorted to self-determined communication strategies such as, (a) the use of hands to support the jaw for producing vocalization, though faint, to draw the attention of the care-givers; and, (b) the use the mobile phone for typing messages he wished to communicate, although his hand movements were restricted.

The patient attended 8 speech-language therapy sessions spread over 2 weeks. Each session lasted for 45 minutes. The sessions were targeted to improve the respiratory support for speech production, speech articulatory movements, and oro-motor tasks focussing on lips, tongue, jaw movements and strength. In the sessions, use of breathing strategies (with tactile feedback) were initiated to both help the patient relax and to support phonation. He was instructed to imitate oral configurations and production of vowels as well as bilabial and lingual sounds. He was also encouraged to use the self-determined strategy of texting using a mobile phone. In addition, he was also asked to use a simple communication board, that is, a board with a binary choice of yes/no with all activities of daily living as well as his usual diet (from which he could choose what he wanted for a meal) listed.

At the end of 8 sessions, at the time of discharge, the patient could phonate occasional vowels, bilabials, linguals, and simple bi-syllables. His drooling had significantly reduced. The patient and his family were counselled on the need for continued speech-language therapy.

Cognitive decline and dysarthria are the most frequently reported disabilities in patients with BSPDC. Our patient, however, had anarthria with intact cognitive functions. The self-determined communication strategy as well as adaptability to speech-language therapy and additional communication strategy are a testimony to his intact cognitive functions. This report highlights the need for speech and language evaluation and intervention in such patients with severe dysarthria or anarthria.

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