35 year old man develops: -Hemiparesis -homonymous hemianopia -Cognitive deterioation MRI shows: -Widespread areas of abnormal T2 signal in white matter -EEG shows slowing over both hemispheres -Biopsy shows demyelination with abnormal giant oligodendrocytes some of which ahve eosinophilic intranuclear inclusions 1. What is this condition? 2. What is this condition associated with?

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Neurology is the branch of medicine that focuses on the study, diagnosis, and management of conditions affecting the nervous system.

1. 35 year old man develops: -Hemiparesis -homonymous hemianopia -Cognitive deterioation MRI shows: -Widespread areas of abnormal T2 signal in white matter -EEG shows slowing over both hemispheres -Biopsy shows demyelination with abnormal giant oligodendrocytes some of which ahve eosinophilic intranuclear inclusions 1. What is this condition? 2. What is this condition associated with?

1. Progressive multifocal leukoencephalopathy (PML), caused by JC polyomavirus Infects oligodendrocytes causing demylination Histology shows eosinophilic intranuclear incluesions 2. AIDS
1. Spindle afferents 2. Signal sent back to spinal cord via 1a afferent fibers and synapse on anterior horn cell. Alpha motor neurons are activated = There are two sets of alpha motor neurons. One goes to the homonymous muscle group. In this case the quadracept and this causes contraction to restore muscle to original length. Another alpha motor neuron is stimulated by an inhibitory neuron that is innervated by the 1a afferent neuron of the muscle spindle. This causes relaxation in the opposing muscle. In this case the hamstring. Gamma motor neurons innervate muscle spindles causing them to contract in response to stretch. 3. Myotatic reflex
1. Autosomal recessive neurodegenerative disease. 2. Mutation in ATM gene = impaired V(d)J recombination. 3. Cerebellar atrophy, spider angiomas, IgA deficiency, lymphopenia, elevated AFP. 4. Repair double strand DNA breaks and prevent their replication.
1. Inherited neuropathy causes demyelination of peripheral nerves. 2. Nerve commonly affected is deep fibular nerve. 3. Inability to dorsiflex, high steping gait due to foot drop 4. Stork like legs, pes cavus (high arched feet), sensory loss.

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