Role of Physiotherapy Intervention in Adult Moya Moya Disease - A Single Case Study

Introduction

Moya Moya disease is a progressive stenotic occlusive disease of bilateral carotid bifurcations with the formation of fine collateral networks, with an angiographic diagnosis. Takeuchi and Shimizu in 1957 first named the disease in Japan.¹ Moya Moya is indicated for Moya Moya syndrome (MMS) or quasi Moya Moya disease (individuals with well-known co-morbidities and angiographic evidence of unilateral/ bilateral stenosis) and Moya Moya disease (individuals without MMD, associated disorders and bilateral stenosis).² It is a rare cerebrovascular disease with an incidence of 0.086 per 100,000 population.³ Incidence of MMD is higher in East Asian countries and lower in European and North American countries.⁴ Aetiology of this is still unknown. Its sites of origin are at the ends of the internal carotid (ICA) on both sides. These small multiple vessels at the base of the brain are known as "moya moya vessels". This condition was observed by catheter angiography and was named ‘Moya Moya’ as it means “cloud of smoke” or “puff of smoke” in Japanese. MMD has a peak age of appearance, 2/3rd of cases occur in children and 1/4th of cases are seen in adults at the age of 40. In general, ischemic stroke occurs in the pediatric group and hemorrhaging stroke occurs in adults.

Case Presentation

A 33-year-old female engineer turned soft skill trainer, with hypertension, hypothyroidism and type 1 DM, was asymptomatic until March 2021 when she had a sudden onset of holo-cranial headache, non-projectile vomiting and was conservatively managed. After nine months, the severity and frequency of headache and vomiting gradually increased for which she re-consulted a neurosurgeon. Investigations revealed Moya-Moya disease Suzuki grade III for which she underwent a right superficial temporal artery to middle cerebral artery bypass. Post the procedure, she started experiencing left shoulder weakness and mild pain impairing her activities.

Again after five months, the weakness progressed to the entire right upper limb along with deviation of mouth to the left side and slurring of speech. Repeat investigations showed multiple small infarcts in the bilateral frontal and parietal lobes for which she was advised left superficial temporal artery to middle cerebral artery bypass.

This patient presented to Neuro-physiotherapy OPD after four months post-surgery with complaints of right shoulder pain and weakness of the left upper limb with difficulties in her Activities of daily living (ADLs) and occupation. On examination, the patient was conscious, oriented, and cognitively sound with clear speech. The functions of the right trigeminal, vestibulocochlear and spinal accessory nerves were impaired along with light touch over the right anterior arm region.

The gross muscle power for the right shoulder = 3-; right elbow and wrist, lower limb & left UL & LL=4. Tone assessment according to Modified Ashworth Scale for right elbow flexors and wrist supinator was 1 and was normal on the left limb. Superficial plantar reflex B/L =3+ and deep tendon reflex bicep, brachioradialis right side=3+. Posture assessment was done where anterior view deviation was right shoulder elevation, right knee in genu valgum, bilateral flat foot. Posterior view deviations were rounded shoulders. Lateral view deviations were forward neck posture, rounded shoulders and increased lumbar lordosis. Gait, balance and coordination were intact.

The brain MRI showed emboli in the outer watershed area of bilateral middle cerebral artery-posterior cerebral artery (MCA-PCA) territories, giving small acute infarcts, and severe luminal narrowing of the right ICA from bifurcations up to the branching point. The second MRI after one year showed sub-acute infarcts in bilateral frontal and parietal lobes. The patient was on Ecosprin, Thyronorm, Glucomet-GP3 and Ryxodeg injection.

Discussion

This case report of adult-onset Moya Moya disease aimed to highlight the protocol of eight weeks. The patient was assessed using SPADI, and DASH and instability was observed in the shoulder, with reduced range of motion, and reduced muscle power. To improve the range of motion, the movement with mobilization (MWM) mulligan concept was administered weekly thrice. MWM has a direct effect on peripheral mechanics to reduce nociceptive mechanoreceptor activation and increase tactile and proprioceptive mechanoreceptors activation. The indirect effect on central mechanisms include increased descending inhibition, uncoupling of pain and movement, and reduced fear of movement. Physiotherapy rehabilitation may improve the functional status and reduce the disability in Moya Moya disease.

In contrast to the previous pediatrics study by Parmar Sanjay et al., this single case study showed development in multisystem deficits like cognitive, visual, motor and other barriers. As per the literature, very few studies explained the role of physiotherapy programs and they concluded that maintenance therapy plays a major role in Moya Moya disease.¹

Damayanti Sethy et al. stated that a circuit training program on walking ability in a 9-year-old female child helped to improve muscle strength of the lower limb, walking speed and endurance with Moya Moya disease.⁷

In conclusion, an evident improvement was seen in shoulder pain and shoulder muscle strength at the end of eight week protocol. Patient could independently perform activities of daily living without any pain which was beneficial in rehabilitating the individual with Moya Moya disease.

Conflict of interest

Nil