Türk Nöroloji Dergisi (Feb 2005)
Amyotrophic Lateral Sclerosis, A Neuropathological Study
Abstract
Scientific Background: Amyotrophic lateral sclerosis (ALS) is a neurodejenerative disorder of unknown cause characterized by degeneration and death of motor neurons. The diagnosis requires clinical evidence of both upper and lower motor neuron dysfunction and diagnostic tests to exclude other disorders with similar symptomatology. Pathologically ALS is characterized by loss of motor nerve cells in the spinal cord, brainstem and primary motor cortex accompanied by degeneration of the corticospinal tracts. A variety of inclusions have been described as major pathological features in ALS and other neurodegenerative disorders, these inclusions clarify the diagnosis and can provide insight into biochemical pathways leading to cell death. Objectives: This study is a review of the clinical and neuropathological features of sporadic ALS with special attention on two of the most common inclusions; Bunina bodies and ubiquitin immuonreactive (ub-ir) inclusions. Material and Methods: We retrospectively analysed the clinical features of 11 autopsy confirmed ALS patients and classified the patients according to the revised El Escorial criteria. We also evaluated the motor cortex, hypoglossal nucleus and spinal cord sections from autopsies of these ALS patients and 7 controls, focusing on the specificity of Bunina bodies and ub-ir inclusions. Results: 4 patients qualified for clinically probable ALS, 1 patient for clinically probable-laboratory supported ALS, 5 patients for progressive spinal muscular atrophy and 1 patient for progressive bulbar palsy. Both Bunina bodies and ub-ir inclusions were found in most of these cases, %81.8 and %90.9 respectively. No Bunina bodies were seen in the control cases while ub-ir inclusions were seen in 3 control cases. Conclusions: This study confirms the specificity of Bunina bodies in sporadic ALS. Since ub-ir inclusions were also seen in a few of the controls, although they are characteristic of sporadic ALS they are not entirely specific.
