Objective: To analyze the dysarthria and speech diadochokinetic rate in a patient with hereditary spastic paraplegia (SPG3A).

Background: Hereditary spastic paraplegias (HSP) are about 80 disorders characterized by spasticity after corticospinal tract dysfunction. ^1-2 They affect about 2-8 per 100,000 people worldwide. ^3-4 SPG3A is a rare neurodegenerative disorder caused by a mutation in the ATL1 gene. Its symptomatology includes progressive spasticity, weakness in the lower extremities, diminished vibration sense, and dysarthria. ⁵ Its rate of progression is slow. Sometimes, the patients are wheelchair dependent or need a walking aid (cane, walker, or wheelchair).

Method: The speech rate of two Greek-speaking brothers with SPG3A (patient one age = 34.5 years; patient two age = 35.8 years) and a pair-matched male control (age = 34 years) was recorded through electroglottography (EGG) ^6,7 and analyzed using the Praat software program for acoustic speech analysis. ⁸ Then, an acoustic analysis for the rapid repetition of the syllables /pa/, /ta/, /ka/ (Alternating Motion Rates, AMRs), and /pataka/ (Sequential Motion Rate, SMR) was taken place. EGG has been used for recording and measuring dysarthrophonia in many neurological diseases. ^9-12

Results: The clinical examination revealed that the first patient’s speech was characterized by mild spastic dysarthria and slowness, while the second was characterized by moderate spastic dysarthria and slowness. The patients’ quantitative speech analysis showed a two to four increase in the mean duration of the syllables /pa/, /ta/, /ka/, and the mean duration of /pataka/ compared to the control participant. Also, the mean time of the /a/ sound in the syllable /ka/ was longer in the patient with moderate dysarthria (patient two = 0.631 milliseconds, patient one = 0.181 milliseconds, control = 0.088 milliseconds). Consequently, the marker for spastic dysarthria in these patients with ATL-1 gene hereditary spastic paraplegia was the /ka/ syllable production.

Conclusion: This is the first quantitative analysis of speech rate in a patient with SPG3A. Quantitative speech analysis can help assess speech rate and monitor speech therapy changes in common and rare neurological diseases.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/quantitative-analysis-of-speech-diadochokinetic-rate-in-hereditary-spastic-paraplegia-spg3a/