Here's another report from the conference. I'm posting this one, since I think that it might be important to many of us. I for one, have had a neurologist misdiagnose me with MS and then seem very concerned when my MRI was normal. This presentation made it all make sense to me.
Neuropathy in SJS and other rheumatic diseases Ann Parke, MD
Dr. Parke is from the Univ. of Connecticut Health Center. She says that rheumatic diseases are systemic ? arthritis and skin complaints are just some of the symptoms.
Neurological symptoms may occur in The Central Nervous System (brain and spinal cord), The peripheral Nervous System (sensory somatic system) or The Autonomic Nervous System.
Sensory neurons conduct messages from the external environment to the CNS. Motor nerves conduct messages from the CNS to muscles and glands.
The Autonomic system consists of sensory and motor neurons that connect the CNS to the organs and conduct mostly involuntary messages (breathing, etc.) and use two groups of motor neurons ? one pre, one post ganglionic. Ganglia are on either side of spinal cord.
Simple entrapment syndromes ? carpal tunnel syndrome or radiculopathies
Inflammatory ? vasculitis and/or auto antibody a. Ro antibody ?pure sensory neuropathy associated with SSA antibody - 2% SJS patients. B. Phospholipid antibody thrombotic and demyelinating syndrome ? 14% SJS patients ? more common in Lupus.
Vasculitis ? purpura ? red , raised dots on legs, upper thighs, or trunk
? Central disease includes stroke-like symptoms, aseptic meningitis, dementia, demyelination syndrome, acute psychosis and sudden acute hearing loss.
? Peripheral Disease ? mononeuritis multiplex ? damage to nerves outside the brain and spinal cord ( added info - http://emedicine.medscape.com/article/316024-overview
Peripheral Neuropathy - symptoms depend on underlying pathology and size of affected nerves. Large nerve abnormalities can be detected by nerve conduction tests. Skin biopsies (epidermal nerve fiber density biopsy) can detect small fiber disease. Nerve biopsies help diagnose vasculitis.
? Commonly a sensory neuropathy
? Usually lower limbs
? Glove and stocking pattern
? Usually mild
? Combined mixed polyneuropathy is more severe.
? Burning pain & tingling
? Lancing pain
? Usually in feet & legs
Up to 60% SJS patients may have PN, mostly small fiber neuropathy.
Cranial Neuropathies ? Bells palsy, trigeminal neuralgia and others
? Tilt table tests show abnormalities ? blood pressure not well maintained
? Mixture of sympathetic (fight or flight system) and parasympathetic (returns body to normal) dysfunction.
? Evidence shows that there is abnormal neuro-endocrine control
? May be caused by inflammatory cytokines ? interfere with neural release of acetyl choline
SJS ? antibodies to muscarinic 3 receptor have been shown in SJS ? interfere with glandular function in animal models.
SLE - autoantibodies associated with other diseases and syndromes:
? Protein-Phosphilipid antibody ? recurrent arterial & venous thrombosis
? Antimitochondrial antibody ? Primary biliary cirrhosis
? Anti Ro antibody ? Neonatal lupus syndrome
? A.N.C.A. ? Wegners Granulomatosis, other vasculitidies
Phospholipid antibody syndrome ? cerebral ischemic disease
Autoantibodies and Disease - autoantibodies may be present for years before clinical complaints develop. Loss of tolerance and autoantibody production is genetically controlled and environmentally triggered.
New meds on the horizon ? those that interfere with abnormal B cell function.
I've seen so many people asking questions about neuropathies, so I hope that this report is helpful.