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Amylotrophic Lateral Sclerosis:Differentiation from Surgical Disease : 

Amylotrophic Lateral Sclerosis:Differentiation from Surgical Disease Robert Ayer,MD

Outline : 

Outline Presenting Symptoms Epidemiology Etiology Differential Diagnosis Differentiation from Cervical Spondylotic Myelopathy Confirming the Diagnosis of ALS

Key Clinical Features : 

Key Clinical Features Voluntary Muscles are Involved Spares the voluntary eye muscles and urinary sphincter muscles A mixed upper a lower motor neuron disease UMN-mild spasticity LMN-atrophy and fascilutions Classical Presentation: Weakness and Atrophy of the hands Spasticity and hyperreflexia of lower extremities +/- Dysphagia and Dysarthria from involvement of brainstem motor nuclei. Tongue Fasiculations No Sensory, or Autonomic Dysfunction 1-2% Incidence of associated dementia

Epidemiology : 

Epidemiology Most Frequent Adult Onset Progressive Motor Neuron Disease Onset ~55 years 50% Dead in 3 years 90% Dead in 6 years Leading Cause of Death is Respiratory Failure Pneumonia Decubitus Ulcers, Skin Infections

Pathophysiology : 

Pathophysiology Degeneration of: Alpha motor neurons of spinal cord Upper Motor Neurons and corticospinal tracts Mechanisms unknown, but areas of investigation include Loss of Growth Factor Support: Decreasaed CSF IGF-1 Excitototcity: Abnormal EAAT-2 expression, a glutamate receptor Oxidative Stress: SOD-1 mutations in 20% of familial cases. Chromosome 21 is location of SOD-1 mutation. Bunina Bodies: neuronal intracytoplasmic eosinophilic inclusion bodies specific for ALS.

Differentiation from Cervical Spondylotic Myelopathy : 

Differentiation from Cervical Spondylotic Myelopathy Cervical Spondylosis: Degenerative conditions of the cervical spine producing 3 main types of clinical problems Nerve Root Compression-Radicular Symptoms Spinal Cord Compression-Myelopathy Pain and Paresthesia of the head,neck,shoulders. Leading cause of myelopathy in Patients >55 yrs.

Cervical Spondylotic MyelopathyClinical Symptoms : 

Cervical Spondylotic MyelopathyClinical Symptoms Time Course: 75% of patients have a progressive deterioration 25% plateu after and intial decline that may remain stable for years Motor: UMN signs from Cord Compression Spasticity in the upper extremities LMN signs from nerve root compression muscle wasting in the upper extremities. Sensory: Sensory level present 41% Dermatomal sensory loss UE 33% Glove distribution losses in hands or feet may occur. Reflexes: Hyperactive 80% at varying distances below the stenosis Sphincter: Urinary urgency common.

ALS vs Spinal Cord StenosisKey Distinguishing Features : 

ALS vs Spinal Cord StenosisKey Distinguishing Features ALS Sensory Changes are absent Sphincter control is maintained Brainstem pathology is present Dysarthia Hyperactive Jaw Jerk Reflex LMN signs in the tongue-Fasiculations LMN signs in the legs Cervical Stensosis: Neck and shoulder pain LMN sign restricted to 1 or 2 spinal cord segments.

Supplementary Tests for ALS Diagnosis : 

Supplementary Tests for ALS Diagnosis EMG: Fibrillations LE fibrillations in the absense of lumbar stenosis Fibrillation potentials in the tongue LP: CSF may demonstrate elevated proteins

J.G. : 

J.G.

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50 y/o male w 2y hx of progressive BLE weakness. Patient complains of significant hand weakness and is currently unable to hold objects. Pt is able to walk put reports weakness in his RLE. He also complain of progressive difficulty with the pronunciation of his words, and difficulty holding his head upright. PMHx: Hypertension and Hyperlipidemia Fam Hx: May have had an uncle with weakness problems. PE: NAD CN II-XII intact. Fasciculation noted on his tongue. Marked atrophy of BLE, with atrophy most notably in his hands. BLE are areflexic BLE are motor intact. Reflex 3+ in BLE. BUE Babinski, and clonus @ ankles. Sensation in Grossly Intact, and no Bowel or bladder incontinence is reported. Spines are non-tender to palpation.