Extrapyramidal involvement in amyotrophic lateral sclerosis: backward falls and retropulsion

Abstract

Three patients with sporadic amyotrophic lateral sclerosis (ALS) presented with a history of backward falls. Impaired postural reflexes and retropulsion accompanied clinical features of ALS. Hypokinesia, decreased arm swing, and a positive glabellar tap were noted in two of these three patients. Cognitive impairment, tremor, axial rigidity, sphincter dysfunction, nuchal dystonia, dysautonomia, and oculomotor dysfunction were absent. Brain MRI disclosed bilateral T2 weighted hyperintensities in the internal capsule and globus pallidus in one patient. Necropsy studies performed late in the course of ALS have shown degeneration in extrapyramidal sites-for example, the globus pallidus, thalamus, and substantia nigra. Clinically, backward falls and retropulsion may occur early in ALS. This may reflect extrapyramidal involvement. (J Neurol Neurosurg Psychiatry 1999;67:214-216) Keywords: amyotrophic lateral sclerosis; backward falls; retropulsion; globus pallidus We found backward falls and retropulsion due to postural instability early in the course of the illness in three of a consecutive series of 22 patients with sporadic amyotrophic lateral sclerosis (ALS). We report the clinical, laboratory, and neuro-imaging features and discuss the possible pathogenic basis of these extrapyramidal features. PATIENT 1 A 60 year old man, previously well, developed slurred speech. He complained of stiVness in both legs and frequent leg cramps. A month later while opening a door he fell backwards and incurred a minor scalp injury. Subsequently, he fell backwards twice while arising from a chair. He noticed frequent twitching of muscles in his arms. Six months later, he developed diYculty in swallowing food and sometimes choked on drinks. He took an increasingly longer time to complete daily activities such as dressing and bathing. There was no history of cognitive decline or tremor. Sensory, sphincter, visual, and autonomic functions were normal. There was no family history of neurological illness. Case reportsGeneral examination and vital indices were normal. There was no postural hypotension. There was a spastic dysarthria, and the palate moved poorly. The jaw jerk was brisk and the tongue was wasted. Fasciculations were seen in the tongue and limb muscles. There was asymmetric wasting in the distal muscles of both upper limbs. Power was 3/5 (MRC) distally, and 4/5 proximally in the upper limbs. In the legs, power was 4/5 in all muscles. Spasticity was found in all four limbs, with mild cogwheel rigidity at the wrists. There was hypokinesia of the face and posture. The speed of rapid rhythmic finger and toe tapping was reduced but there was no rest tremor. Postural reflexes were impaired. When gently pulled backwards, even after a prior warning, he could not maintain stability and toppled backwards. There was no adaptation to repetition of this manoeuvre. A gentle push on the chest resulted in a backward fall. He could maintain an upright stance and did not fall on being pushed forwards or laterally. All deep tendon...

The phenotypic variability of amyotrophic lateral sclerosis

“…Mild parkinsonism is present in 5-15% of patients with ALS, and is typically characterized by postural instability and backward falls. 78,81 In such patients, abnormalities in the nigro striatal system can be seen on dopamine transporter imaging or at autopsy. 78,[81][82][83] In our experience, distinguishing the spastic, extrapyramidal and frontal motor findings in such patients is not trivial, which could explain why different studies have reported variable frequencies of ALS-parkinsonism.…”

Sporadic Parkinson disease and Amyotrophic Lateral Sclerosis complex (Brait–Fahn–Schwartz Disease)

“…Symptoms and signs of parkinsonism have also been described in ALS [2,4,6,[10][11][12], with a frequency ranging from 5% to 17% [12][13][14]. ALS however is a distinct MND, which involves both upper and lower motor neurons-related symptoms and signs, and it shows a steadily clinical progression.…”

“…Extrapyramidal signs and symptoms due to nigrostriatal dysfunction have been reported in patients with Amyotrophic Lateral Sclerosis (ALS) [1][2][3][4][5]. However, outside the known ALS-Parkinson-Dementia complex (ALS-PD) of the Guam and Kii Peninsula of Japan, the comorbidity of Parkinson's disease and ALS is rare.…”

Amyotrophic lateral sclerosis: Clinical heterogeneity and approaches to classification

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