10, 11 Other series have described bulbar onset then skipping the arms to appear in the legs. In one series an ascending progression was noted in limb onset patients, with progression from one side to the other occurring first, followed by ascending progression (average 3.5 years from onset to arm involvement, 5 years from onset to bulbar involvement). Patterns of progression most commonly show spread from side to side, and from region to region, with many patients ultimately developing spastic quadriparesis with bulbar involvement. Case reports have described cognitive changes in cases termed PLS plus, or overlap with Parkinsonian syndromes. 2, 3, 7 Generally cognition is reported as being unaffected in PLS however some frontal lobe dysfunction can be seen in 10–20% of patients. 1, 3 Urinary frequency or urgency can be seen in around 1/3 to half of patients. 6 An upper motor neuron pattern of weakness may be seen (extensors in upper extremity, flexors in lower extremity), but what the patient describes as weakness is often a combination of increased tone, decreased coordination, and mild weakness.Īlthough visual symptoms are not reported some abnormalities of eye movements have been described, including saccadic breakdown of smooth pursuits, or supranuclear paralysis. Stiffness as a presenting symptom is seen more commonly in PLS than ALS (47% versus 4%), and limb wasting is rare in PLS (~2%). Typically the examination shows only upper motor neuron signs, spasticity, spread of reflexes, and absence of lower motor neuron findings (fasciculations and muscle wasting). Bulbar symptoms can include dysarthria, dysphagia, and emotional lability (fits of laughing or crying, termed pseudobulbar affect). Most patients will report balance difficulties, and as the disease progresses increasing falls. 5 Patients may report stiffness, clumsiness and poor coordination. The most common clinical presentation matches Erb’s original description of patients with spastic spinal paralysis from the early 20 th Century, which included spasticity, hyperreflexia, and mild weakness. As is seen in ALS there may be a slight male predominance. 4 The vast majority of patients present > 20 years of age. The prognosis in PLS is more benign than ALS, making this a useful diagnostic category.Īlthough considerable heterogeneity exists between patients, symptoms usually begin in the 5–6th decade, unlike hereditary forms of spastic paresis which usually present earlier and are associated with foot deformities which are not present in PLS. Treatment is largely supportive, and includes medications for spasticity, baclofen pump, and treatment for pseudobulbar affect. Although no test is specific for PLS, some neurodiagnostic tests are supportive: including absent or delayed central motor conduction times and changes in the precentral gyrus or corticospinal tracts on MRI, DTI or MR Spectroscopy. EMG is normal, or only shows mild neurogenic findings in a few muscles, not meeting El Escorial criteria. The diagnosis is made based on clinical history, typical exam findings, and diagnostic testing negative for other causes of upper motor neuron dysfunction. Patients experience stiffness, decreased balance and coordination, and mild weakness, and if the bulbar region is affected, difficulty speaking and swallowing, and emotional lability. Primary lateral sclerosis (PLS) is characterized by insidious onset of progressive upper motor neuron dysfunction in the absence of clinical signs of lower motor neuron involvement.
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