RESUMO
OBJECTIVE: To investigate swallowing characteristics of patients with dysphagia caused by anterior cervical osteophytes (ACOs) and compare clinical courses according to treatment options. METHODS: A retrospective analysis of 1,866 videofluoroscopic swallowing studies (VFSS) of patients with ACOs from electronic medical records was performed. Patients with other diseases that could explain the dysphagia were excluded. Dysphagia characteristics and severity and clinical and radiological characteristics of subjects with ACOs were evaluated. Dysphagia characteristics and clinical course were compared among three treatment groups: surgical treatment, swallowing rehabilitation, and conservative treatment. RESULTS: Subjects were 22 men and 1 woman with a mean age of 78.69±8.01 years. The mean osteophyte thickness was 9.07±3.84 mm. It was significantly thicker in the surgical group than that in other groups (p=0.01). ACOs were most frequently found at C5 level. This level also had the thickest osteophytes. However, videofluoroscopic dysphagia scales (VDS) were not significantly different among the three treatment groups. The pharyngeal phase score of the VDS was significantly higher in the surgical group (p=0.041). Dysphagia severity was decreased significantly in the surgical group at 3 months after the initial VFSS (p=0.004). CONCLUSION: The main swallowing characteristics in patients with ACOs were dysphagia features of the pharyngeal phase, including inappropriate airway protection, decreased laryngeal elevation, and reduced epiglottis inversion. When determining treatment options, it may be helpful to consider dysphagia severity at pharyngeal phase and osteophyte thickness.
RESUMO
Pantothenate kinase-associated neurodegeneration (PKAN) is a neurodegenerative disorder characterized by iron accumulation in the globus pallidus (GP) of the brain (neurodegeneration with brain iron accumulation [NBIA]), which is characterized by dystonia and spasticity resulting in postural difficulties. A 33-month-old boy was admitted with a pronounced gait disturbance. Marked hypertonicity in the patient's both calf muscles was noted, resulting in waddling with repeated slip-falls. NBIA was suspected by high T2 intensity in the GP on brain MRI, then it was confirmed by detecting PANK2 mutation. Botulinum toxin-A injection was administered to both calf muscles. After 2 weeks, a decrease in spasticity and an increase in range of motion were observed, and consequently, an increase in the patient's gait stability with both heels touching the ground, enabling him to walk straight independently. A definitive treatment for NBIA has not been established, and a symptomatic therapy is currently the mainstay of treatment in this case. This is the first case report of botulinum toxin injection for treatment of gait disturbance caused by spasticity in an infantile-onset PKAN.
