A multidisciplinary approach to assess recovery of consciousness in a patient with moyamoya disease

Abstract Introduction Moyamoya disease (MMD) meaning “hazy puff of smoke” in Japanese is a rare chronic cerebrovascular syndrome characterized by progressive stenosis and occlusion of the internal carotid arteries (ICAs) anterior cerebral arteries (ACAs), and middle cerebral arteries (MCAs). Methods Our moyamoya patient with severely impaired cognitive and motor functions underwent a specific motor and neuropsychological rehabilitative treatments to assess the recovery of consciousness. Results Results obtained showed an improvement of clinical and neuropsychological examination. These findings highlighted the importance of an intensive rehabilitation techniques used in the care of disorders of consciousness patients. Conclusions The use of sensory methods provides advantages for the rehabilitation. In fact, in this study, we showed a correlation between sensory stimulation and changes in patient's clinical status.

motor functions. The patient underwent multidisciplinary rehabilitative program in order to demonstrate the importance of sensory and cognitive rehabilitative techniques and to assess the possible recovery of consciousness.

| C A S E DE SCRIP TI ON
We report a case of 31-year-old right-handed male patient. He came to our neurorehabilitative care unit after hemorrhagic cerebral event. He presented a severe medical history. He was submitted to craniotomy for decompression of hematoma in fronto-parietal right regions. He also presented devices for nutrition (PEG) and for spontaneous breath (Tracheal Cannula). Medical management included Baclofen, Ramipril and Levodopa/benserazide cloridrato. All these clinical aspects were compatible with MMD During hospitalization, the patient in our institute, performed a specific motor, logopedic, neuropsychological rehabilitative treatments.
At first, we immediately conducted a neuropsychological and clinical evaluation. Patient was evaluated as a Vegetative State (VS).
For this reason, it was impossible to undertake a global neuropsychological evaluation. Patient was awake, but verbal and nonverbal communication were absent. There was no contact with external environment. We used clinical scales to evaluate evolutions of consciousness's state such as: Coma Recovery Scale-Revised (CRS-R), Level of Cognitive Functioning (LCF), Disability Rating Scale (DRS), and Glasgow Coma Scale (GCS). He was re-evaluated at least 1-2 times/week, to monitor conscious improvements (Table 1).
Patient was submitted to magnetic resonance angiography that showed several parenchymal supra-and subtentorial brain arteriovenous malformation (AVMs) with "smoke cloud" evident in dif- We used Neurowave System to perform multisensory stimulation and analyze biophysical parameters (De Salvo, Caminiti et al., 2015;De Salvo, Naro et al., 2015). The ERPs recorded, showed moderate improvement between T0-T1. At T0, the P300 wave was absent; while at T1, the P300 wave was present even if the latency and amplitude parameters were significantly altered.
The multidisciplinary team assessed the patient everyday: he was treated for logopedic, physiotherapeutic, and neuropsychological rehabilitation. Physiatrist, in collaboration with neurologists, neuropsychologists, and therapists (logopedists and physiotherapists), planned a rehabilitation program. Members of the patient's family were updated about clinical conditions and the progress of the rehabilitation program every month.
We started rehabilitative treatment 1 month after acute event and was carried out for 6 months (six times a week). Patient was treated two times daily for 60 min to each session. The logopedists, in particular, contributed to tracheostomic cannula with respiratory and phonatory exercises. Physiotherapeutic treatments included passive loosening exercises of upper and lower limbs, manipulation exercises to reduce spasticity, and exercises to improve trunk control. Neuropsychologists used devices to improve the auditory and visual tracking.
At the first cognitive evaluation, we observed that auditory channel was more responsive than visual channel. During rehabilitative process, multisensory devices were used to stimulate visual and auditory channels such as Neurowave System and, Sniffin Stick test to stimulate olfactory way (Bonanno et al., 2017) and tactile stimulation to record different tactile responses (hot, cold, rough, smooth, soft). After 2 months of hospitalization, our patient became more responsive by using visual tracking. Our results confirmed that patient had a moderate recovery of consciousness. In fact, clinical scale scores improved especially between the second and third time point (see Table 1). Patient was assessed as a minimal responder because he showed self-awareness of the surrounding environment.
Although, cognitive and relational profile showed a clinical recovery, motor profile remained unchanged.

| D ISCUSS I ON
The natural history of MMD is not fully understood. There are few studies on disease progression. Early clinical investigations demonstrated that in pediatric patients, who were conservatively treated, functional or intellectual outcome was poor. Adult patients with MMD, who have unilateral lesions, were carefully monitored to control the potential progression to bilateral lesions (Kuroda & Houkin, 2008 techniques have been extended also to patients with altered consciousness. In particular, the use of sensorial methods provides several advantages for rehabilitation. Studies demonstrated a correlation between sensory stimulation and changes in patient's clinical status (De Salvo, Caminiti et al., 2015;De Salvo, Naro et al., 2015). Several studies described patients with cognitive dysfunctions treated by a specific cognitive rehabilitative program (Calabrò et al., 2015;Coughlin, Miller, & Schuette, 2017;Takagi & Miyamoto, 2015). The neuropsychological and imaging data obtained from the present case showed that, severe cognitive dysfunctions are really disabling in chronic cerebrovascular disorders, including MMD. In this case, we highlighted the importance of neuropsychological periodic assessment to evaluate the improvement of consciousness. In fact, these findings highlighted the importance of intensive rehabilitative techniques (La Gattuta et al., 2018).
This case demonstrated the evidence of a possible recovery of cognitive and motor functions after a MMD. Early-and long-term rehabilitation can affect the outcome in severe brain injury such as MMD. In this case, multidisciplinary programs and intensive rehabilitation were performed. Indeed, it is possible that the long-term rehabilitation could recover several clinical functions. In addition, a more appropriate model of motor and cognitive multisensory stimulation, programmed by multidisciplinary rehabilitation team, could influence the recovery of consciousness inducing mechanisms of neuronal plasticity.

CO N FLI C T O F I NTE R E S T
The authors declare that they have no conflict of interest.

R E FE R E N C E S
F I G U R E 1 (a) Axial SE T1-weighted (b) coronal TSE T2-weighted images Right capsular, fronto-parietal, and insular lobe hemorrhage. (c) ToF-3D, MIP reconstructions. The image shows some bilateral nidus of vessels through which arteriovenous shunting occurs (a) (b) (c)