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- Congenital neuropathy
- Inflammatory, dysglobulinemic
- Vasculitides
EMGcan distinguish congenital neuropathy from other forms 4 typical reasons to get EMG diagnose neuropathy (versus myopathy, other) characterize axonal versus demyelinating define extent rule out other forms of neuropathy in differential diffuse demyelination: markedly slow sensory nerve conduction velocities and conduction block on nerve conduction studies Note: examination of flexor hallucis brevis muscle may establish motor involvement which is not otherwise apparent (in larger muscles) MRI Congenital neuropathycan be minimal for a long time and then cause joint deformity later on (even beginning at an older age) / other types of neuropathy typically do not cause joint deformity Charcot-Marie-Tooth most common congenital neuropathy / slow progression over years Presentation: “numbness” without prickling or tingling, symmetric weakness on dorsiflexion of feet (“steppage” gait), asymptomatic weakness of hands, and pes cavus with hammer toes / has primary demyelinating form CMT Ia (resembles CIDP) Labs: may have increased CK (non-specific) Diagnosis: EMG (r/o other motor disease), can also do DNA testing for involved genes (commercially available tests) CIDP Labs: anti-MAG (can show up in CSF) Acquired neuropathy DM (see other) symmetric, ANS, focal, multi-focal, axonal and demyelinating usually distal occurs first (longer nerves) cranial and autonomic tends to be more asymmetric and reversible than the distal Inflammatory, dysglobulinemicplasma cell dyscrasias or Castleman’s disease Drug-inducedamiodarone, chloroquine LeukodystrophiesKrabbe’s uremic, carcinomatous, small cell carcinoma of lung, leukemias VasculitidesPAN, RA, SLE, Sjogren’s, Wegener’s Infections HIV, HCV, HBV, HTLV-1, syphilis, lyme, leprosy Carpal Tunnel Syndrome Presentation: numbness/pain in fingers/hands, usually worse w/ wrist flexion (caused by involvement of median nerve) / also very common is compression of lateral femoral cutaneous nerve (from recent wt gain or other), causing numbness in lateral, upper thighs Causes: can be caused by repetitive trauma/overuse, but also can come from any inflammatory process in wrist (with tissue deposition) such as diabetes, hypothyroidism, RA, amyloidosis Physical exam: Tinel’s (tap on palm just distal to flexor retinaculum, elicits numbness/pain if positive), Phalen’s (bend wrists and hold backs of hands together for 1 minute, to check for numbness/pain) Diagnosis: can get EMG to confirm diagnosis, evaluate degree of nerve involvement Treatment: splint wrists at night, reduce causative activity, surgery if medical management fails DeQuervrain’s tenosynovitis focal wrist pain on radial aspect of hand due to inflammation of tendon sheath of abductor pollicis longus / should not have positive Tinel sign or median nerve involvement Treatment: conservative to intra-sheath steroid injection
T4 and L1 are most vulnerable (boundary zones) / aortic aneurysms, atherosclerosis Spinal cord injury fracture is tender to palpation / brisk reflexes below lesion Treatment: NSGY consult, methylprednisolone may be helpful within 8 hrs / vertebral Cervical C6 upper arm, shoulder / index finger and thumb / biceps and brachioradialis C7 arm and forearm / middle finger / triceps Lumbar sciatic pain in both S1 and L5 / Treatment: NSAIDS and rest or surgery if not better in 2-4 weeks and/or radiography reveals nerve root compression L5-S1 (70%) outer aspect of foot / ankle tendon reflex / atrophy of gastrocnemus L4-L5 (25%) outer aspect of leg and dorsum of foot / great toe weakness / foot drop Extramedullary lesions Causes: metastatic (breast, prostate, lungs) primary (meningioma, neurofibroma) hematoma (warfarin) infection (Tb, many others) Cauda equina syndrome loss of sphincter control / numbness in buttocks/back of thighs / weakness, paralysis of dorsiflexion (L4) and toes (L4/5) and plantar flexion (S1) Diagnosis: plain films, bone scan, MRI (depends on situation) Treatment: steroids, urgent neurosurgery consult if cord compromised, urgent radiation-oncology consult if due to tumor effect Prognosis: 10% of patients with paraplegia from neoplasm recover ability to walk Intramedullary lesions Causes: astrocytoma, ependymoma, syringomyelia, vascular infarcts, plaque demyelination (MS) Syringomyelia central canal / loss of pain/temperature bilaterally, sparing of pinprick and proprioception / sacral sparing differentiates from extramedullary Outer spinal injury ipsilateral weakness / contralateral loss of pain/temperature below lesion Myopathy
hypothyroid/hyperthyroid hyper PTH
polyneuropathy of DM steroid myopathy vitamin D deficiency uremic polyneuropathy
Myopathy of systemic disease: heart, lungs, liver Drug-induced myopathy: many Download 3.95 Mb. Share with your friends:
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