Motor Neuron Disease PDF

Motor neuron disease By Shaimaa Shaheen Mohammed Assistant professor of Neurology, Faculty of Medicine, Cairo University.  Motor neuron disease Definition Clinical picture Investigation treatment  Definition:...

Motor neuron disease By Shaimaa Shaheen Mohammed Assistant professor of Neurology, Faculty of Medicine, Cairo University.  Motor neuron disease Definition Clinical picture Investigation treatment  Definition: MND It is a degenerative disease affecting the motor system only (upper or lower motor neurons or both of them).  Epidemiology MND Age of onset: middle or old age. Sex: males> females Onset & course: gradual onset & progressive course.  Etiology MND Idiopathic , degenerative disease Para –neoplastic Genetic  Clinical picture MND Motor affection: bilateral & symmetrical affection of UMN, LMN or both of them. UMN affection only as in: spastic paraparesis LM N affection as in spinal muscle atrophy Combined UM,LM as in amyotrophic lateral sclerosis.  Clinical picture con.. Pure UMN affection: Spinal cord affection: - Spastic paraplegia ( dorsal region). - Spastic quadriplegia (cervical region). Cerebral or brain stem: - Psudo bulbar palsy, exaggerated palatal & pharyngeal reflex. - Quadriplegia. - Exaggerated jaw reflex. - Emotional liability.  Clinical picture con.. Pure LMN affection:(progressive muscular atrophy): it usually affects the A.HCs of the lower cervical region with weakness in UL & signs of LMNL. Cranial nerve affection: true bulbar palsy: - Nasal tonation - Nasal regurgitation of fluids. - Lost palatal & pharyngeal reflex. - No emotional liability. - Tongue wasting & fasiculations.  Clinical picture con.. Combined upper & lower motor neuron (amytrophic lateral sclerosis): - In upper limb: wasting, fasciculation, weakness (LMN signs), hypertonia, hypereflexia (UMN signs). Tonic atrophy. - In lower limb: mainly signs of UMN lesion in addition to minimal fasciculation.  Investigation MND EMG, NC on UL and LL. Para-neoplastic work up.  Treatment Physiotherapy Care of bed ridden Care of respiration Multivitamins Riluezol  Pseudo bulbar palsy Vascular: bilateral stroke. Infection: encephalitis. Neoplastic: midline brain stem tumors. Demyelinating: MS. Degenerative: motor neuron disease.  True bulbar palsy: Vascular: vertebrobasilar insufficiency. Infection: diphteria. Neoplastic : brain stem tumors Degenerative: motor neuron disease.  Differential diagnosis of wasting of small muscles of the hand Wasting due to affection of C8, T1 either due to affection of: 1. Spinal cord segments: poliomyelitis, spinal artery occlusion, transverse myelitis. 2. Root affection: spinal spondylosis, tumors, fractures, Pott’s disease. 3. Brachial plexus injury: Klumpke’s paralysis, thoracic outlet syndrome. 4. Peripheral nerve injury: carpal tunnel syndrome, peripheral neuropathy 5. Muscle affection: distal myopathy of Gower 6. Others: rheumatoid arthritis Thank you