![]() If there are only upper motor neuron findings on examination then Primary Lateral Sclerosis needs to be considered in the differential diagnosis (PLS, see chapter 6). In particular if the onset was insidious and the weakness is painless. If a patient comes in with asymmetric onset of distal weakness or muscle wasting and upper motor neuron findings on examination (brisk reflexes with spread to other regions, Babinski sign, Hoffman reflex, or increased tone) ALS is a main consideration. HSAN, diabetes, GBS, amyloid, porphyria, Fabry'sĪsymmetric distal weakness without sensory loss (NP5) Pattern 9 - Asymmetric proprioceptive loss w/out weakness Pattern 8 * Focal midline proximal symmetric weakness Prox & Distal SMA Distal Hereditary motor neuropathy Pattern 7 * Symmetric weakness without sensory loss Pattern 6 - Symmetric sensory loss & upper motor neuron signsī12 defic Copper deficiency, Friedreich's, adrenomyelone uropathy + UMN – ALS Pure UMN - PLS - UMN – MMN, PMA, MAMA, BAD, LAD Pattern 5 - Asymmetric distal weakness w/out sensory loss Pattern 4 - Asymmetric prox & distal weakness w/sensory loss Multiple – Vasculitis, HNPP, MADSAM, infection Pattern 3 - Distal weakness with sensory loss Pattern 2 - Distal sensory loss with/without weakness Pattern 1 - Symmetric prox & Distal weakness w/sensor y loss Here we describe a pattern approach to identifying motor neuron disease, and clinical features of sporadic ALS. The only FDA approved treatments for ALS are riluzole, which prolongs life by about 3 months, and dextromethorphan/quinidine which provides symptomatic relief for pseudobulbar affect (inappropriate bouts of laughter or crying). Amyotrophic lateral sclerosis (ALS) is a progressive mixed upper and lower motor neuron disorder, most commonly sporadic (~85%), which is invariably fatal. MNDs exist on a spectrum: from a pure lower motor neuron to mixed upper and lower motor neuron to a pure upper motor neuron variant in addition to regional variants restricted to the arms, legs or bulbar region. MND is a clinical diagnosis supported by findings on electrodiagnostic testing, in the absence of other abnormalities on neuroimaging or serological testing. When approaching the patient with suspected motor neuron disease (MND) the pattern of weakness on exam helps distinguish MND from other diseases of peripheral nerves, the neuromuscular junction, or muscle.
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