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...

A girl with neuropsychiatric lupus demonstrated Parkinsonian features soon after commencing risperidone. The single photon emission computed tomography scan showed hyperperfusion of the basal ganglia. Symptoms abated with the addition of dopaminergic agents to immunosuppressive therapy. The literature on juvenile Parkinsonism in lupus has been reviewed.

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The pedunculopontine nucleus (PPN) is a potential target for gait disorders. We report 4 cases of bilateral PPN stimulation in progressive supranuclear palsy (PSP) patients with short-term (6 months) and long-term (18 months) follow-ups. Patients with PSP who had gait disturbances, but were able to walk with or without assistance, were selected. The patients' median age was 64 years and the disease duration 3 years. Bilateral PPN deep brain stimulation (DBS) was performed. The pacemaker was programmed using a bipolar mode and lower frequencies (20-45 Hz). The PSP rating scores (PSPRS) and their gait subscores (No. 25, 26, 27 and 28) along with PSP staging scores were used as primary end points. The total Unified Parkinson's Disease Rating Scale (UPDRS), UPDRS III and the 39-item Parkinson's Disease Questionnaire were considered as secondary end points. Video recordings of the gaits were performed before surgery and at the 6- and 18-month follow-ups. These were retrospectively reviewed by a blinded neurologist for the primary end points. At the 6- and 18-month follow-ups, the median change in PSPRS was from 33 (baseline) to 37.5 and 47, respectively. Similarly, the PSP staging changed from 3 to 2.5 and 3.5, item 25 from 1.5 to 2 and 3.5, item 26 from 2.5 to 2 and 3.5, item 27 from 3.5 to 3 and 3.5 and item 28 from 1.5 to 1.5 and 3. Two patients in the study with the PSP-parkinsonism phenotype experienced improvement in their gait until the last follow-up. Bilateral PPN DBS can be safely performed in PSP patients despite mid-brain atrophy.