3. No evidence of conduction block or other features of primary demyelination. 4. EMG demonstrates evidence of active denervation in the form of fibrillation potentials and positive sharp waves as noted above. The earliest abnormality is fasciculation potentials due to motor unit hyperexcitability/instability that occur prior to motor unit degeneration. TREATMENT 1. Riluzole: a. Two controlled trials have demonstrated that riluzole 50 mg p.o. b.i.d. extends tracheostomy-free survival by 2 to 3 months. Unfortunately, the studies did not find that riluzole improves strength or the quality of life. b. Riluzole is thought to act by inhibiting the release of glutamate at presynaptic terminals. c. Side effects include nausea, abdominal discomfort, and hepatotoxicity. d. Check hepatic function tests every month for 3 months and then every 3 months while on riluzole. Hepatotoxicity is reversible once riluzole is discontinued. 2. Supportive care: a. Despite that the lack of effective therapy to halt or reverse the progression of the disease, there are many therapeutic measures that improve the quality of life in patients with ALS. b. A multimodality approach in treating patients with ALS is essential. c. Patients are seen in clinic at least every 3 months in conjunction with physical, occupational, speech, and respiratory therapy. d. They are also evaluated by psychiatry, gastroenterology, pulmonary medicine, and social workers as necessary. 3. Physical therapy: a. Stretching exercises, passive and active, to prevent contractures. b. Assess gait and needs (i.e., cane, walker, wheelchair). 4. Occupational therapy: a. Patients should be evaluated for adaptive devices (e.g., ball-bearing feeders) that may improve function. b. The patient's home should be evaluated for equipment needs. 5. Dysarthria: a. Patients should be evaluated by a speech therapist. b. Techniques may be given to help patient with articulation. P.196 c. Patients may benefit from various speech augmentation devices and switch- or light-guided scanning computerized devices. 6. Dysphagia: a. Because of the associated swallowing difficulties occurring with bulbar weakness, nutrition becomes impaired. b. High-calorie and protein-concentrated supplementation should be added to diet. c. When dysphagia is severe, a percutaneous endoscopy gastrostomy (PEG) is recommended. Some studies have demonstrated that nutrition by PEG or gastrojejunostomy improves quality of life and survival by a few months. 1. Ideally, PEG placement should be done before FVC falls below 50% to reduce the risks of the surgical procedure. 2. PEG placement does not prevent aspiration. 7. Salivation: a. Drooling and hypersalivation can be a problem secondary to swallowing difficulties. b. TCAs [e.g., amitriptyline 10—100 mg p.o. at bed time (qhs)] have anticholinergic properties that can reduce secretions. In addition, patients not uncommonly have a reactive depression that may be helped by the addition of an antidepressant. c. Other medications that can be used include: 1. Glycopyrrolate 1 to 2 mg p.o. b.i.d. to t.i.d. 2. Benztropine 0.5 to 2.0 mg every day (qd) 3. Trihexyphenidyl hydrochloride 1 mg qd to 5 mg t.i.d. 4. Atropine 2.5 mg qd to 5 mg t.i.d. 8. Thick mucus production: a. Some patients describe thick mucus, particularly when using the above medications to treat hypersalivation. b. Beta-blockers such as propranolol and metoprolol may help. c. Acetylcysteine 400 to 600 mg p.o. qd in one to three divided doses or as a nebulizer treatment (3—5 mL of 20% solution every 3 — 5 hours). 9. Spasticity: a. Baclofen 5 mg p.o. t.i.d. to start. May increase up to 80 mg qd (20 mg q.i.d.) as tolerated and as needed. b. Tizanidine 2 mg t.i.d. to start. May increase up to 12 mg t.i.d. as tolerated and as needed. c. Diazepam 2 mg b.i.d. May increase up to 10 mg q.i.d. as tolerated and as needed. 10. Pseudobulbar affect: a. An antidepressant medication can be used, particularly in patients with underlying depression. b. Amitryptiline 10 to 25 mg qhs increasing to 100 mg qhs as necessary. 11. Constipation a. Constipation may result from weakness of the pelvic and abdominal muscles, diminished physical activity, anticholinergic and antispasticity medications, and opioids. b. Management includes increasing dietary fiber and fluid intake, adding bulk-forming laxatives, and using suppositories or enemas as needed. 12. Ventilatory failure: a. Most patients with ALS die as a result of respiratory failure; therefore, it is important to assess for symptoms of signs of respiratory impairment during each clinic visit. b. Patients with forced vital capacities below 50% or those with symptomatic respiratory dysfunction are offered noninvasive ventilator support, usually BiPAP. c. Inspiratory and expiratory pressures are titrated to symptom relief and patient tolerability. d. In my experience, only a few patients desire tracheostomy and mechanical ventilation, because it prolongs expensive and often burdensome care for the P.197 family. However, this is an individual decision that must be made by the patient. Tracheostomy needs to be offered to patients along with realistic counseling in regard to what this entails to the patient and the family. e. Intermittent dyspnea and the anxiety that accompanies it may be treated with lorazepam 0.5 to 2 mg sublingually, opiates (e.g., morphine 5 mg), or midazolam 5 to 10 mg intravenous (IV) (slowly) for severe dyspnea. f. Constant dyspnea can be managed with morphine starting at 2.5 mg q4h or continuous morphine infusion plus diazepam, lorazepam, or midazolam for associated anxiety. g. Thorazine 25 mg every 4 to 12 hours rectally or 12.5 mg every 4 to 12 hours IV should be considered for terminal restlessness. 13. Pain: a. Pain occurs in at least 50% of patients due to muscle cramps, spasticity, limited range of motion and contractures related to weakness, and skin pressure secondary to limited movement. b. Careful positioning and repositioning of the patient, physical therapy to help prevent contractures, antispasticity medications, antidepressants, nonsteroidal antiinflammatory medications, and opioids may be used to treat pain. 14. Psychosocial issues: a. Depression is not uncommon for patients and family members. b. Patients and family members may benefit from local support groups. c. Antidepressant medications. Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition ACUTE POLIOMYELITIS Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND 1. Poliomyelitis is very uncommon in industrialized nations due to routine use of the polio vaccine. 2. However, not everyone is vaccinated, plus a poliomyelitis-like illness can be seen with other viruses (e.g., Coxsackie virus, West Nile virus). PATHOPHYSIOLOGY 1. The virus gains access to the host usually through oral or respiratory route. The virus proliferates and viremia ensues. 2. The virus is taken up into the peripheral nervous system via binding to receptors and the distal motor nerve terminals. 3. Subsequent transport to the anterior horn cell in the spine occurs with degeneration of motor neurons. PROGNOSIS The degree of recovery is variable. Some patients develop weakness and achiness in muscles previously affected (postpolio syndrome, see below). DIAGNOSIS Clinical Features 1. Most people (98%), especially children, experience a minor nonspecific systemic illness for 1 to 4 days: sore throat, vomiting, abdominal pain, low-grade fever, easy fatigue, and minor headache. P.198 2. A small percentage (2%) of individuals develop neck and back stiffness, fasciculations, and asymmetric weakness involving the extremities and/or bulbar musculature. 3. Following the initial illness and paralysis, recovery of function to varying degrees occurs over the ensuing 4 to 8 years. Laboratory Features 1. CSF examination usually reveals increased protein and pleocytosis initially consisting of both polymorphonuclear leukocytes and lymphocytes and then later predominantly lymphocytes. The cell count is usually less than 100 cells/mm 3 . 2. Diagnosis may be confirmed by culture of the offending virus, although the sensitivity is low. Also acute and convalescent antibody titers can be obtained. Electrophysiologic Findings 1. Sensory NCSs are normal. 2. CMAP amplitudes may be reduced in patients with profound muscle atrophy. 3. The motor conduction velocities and distal latencies are normal or slightly abnormal in those individuals consistent with the degree of large fiber loss. 4. EMG demonstrates reduced recruitment of MUAP early with positive sharp waves and fibrillation potentials within 2 to 3 weeks following the onset of paralysis. TREATMENT 1. There is no specific treatment other than supportive care. 2. Respiratory status needs to be monitored closely and patient mechanically ventilated if necessary. 3. Nutritional support if patient is unable eat on his or her own. 4. Physical and occupation therapy are essential to improve function. 5. An antiepileptic medication (e.g., neurontin) or antidepressant medication can be used to treat associated pain that frequently accompanies the acute illness. Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition POSTPOLIOMYELITIS SYNDROME Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND As many as 25% to 60% of patients with a history of poliomyelitis infection develop subsequent neuromuscular symptoms 20 or 30 years after the initial acute attack PATHOPHYSIOLOGY It is thought that motor neurons unaffected by the poliomyelitis sprout to reinnervate previously denervated muscle fibers. These motor units that are increased in size may be under increased stress compared with normal motor units, leading to gradual degeneration over time in some. P.199 PROGNOSIS The course and the symptoms are highly variable but as a rule, actual muscle weakness is slowly progressive, if at all. DIAGNOSIS Clinical Features 1. Patients with postpolio syndrome complain of progressive fatigue (80% — 90%), multiple joint pains (70% — 87%), and muscle pain (70%—85%). 2. Fifty percent to 80% of patients also develop progressive loss of strength and muscle atrophy. This progressive weakness usually involves previously affected muscles but muscles thought to be clinically spared at the time of the acute infection may at times become affected. 3. Muscle cramps and fasciculations are also commonly noted. Laboratory Features 1. Unlike acute poliomyelitis, the CSF does not demonstrate pleocytosis or viral particles. 2. Serum CK levels may be mildly elevated. Electrophysiologic Findings 1. Sensory NCSs are normal. 2. CMAP amplitudes may be reduced in patients with profound muscle atrophy. 3. The motor conduction velocities and distal latencies are normal or only slightly abnormal proportionate to the degree of large fiber loss. 4. EMG demonstrates active denervation in the form of positive sharp waves and fibrillation potentials, fasciculation potentials, and reduced recruitment of long-duration, large-amplitude, polyphasic, unstable MUAPs. TREATMENT 1. There are no specific therapies for postpolio syndrome. 2. Treatment is supportive similar to that for other motor neuron disorders. 3. Physical and occupational therapy can be beneficial. 4. A recent double-blind, placebo-controlled trial demonstrated no benefit with pyridostigmine. 5. Muscle pain may ease with TCA medications. 6. Severe dysphagia, dysarthria, and respiratory weakness are treated as discussed in the ALS section. Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition STIFF PERSON/STIFF LIMB SYNDROME Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND 1. Moersh and Woltman were the first to describe 14 patients with the disorder, which they termed “ stiff man syndrome. ” 2. Because the disorder is more common in women than in men, stiff person syndrome (SPS) has become the preferable name for the disorder. P.200 3. Some authorities have clinically subdivided SPS into three subdivisions: a. Progressive encephalomyelitis with rigidity, b. Typical SPS, and c. Stiff limb syndrome. 4. There is an increased incidence of insulin-dependent diabetes mellitus (IDDM) and various autoimmune disorders. 5. There are reports of SPS associated with Hodgkin lymphoma, small cell carcinoma of the lung, and cancers of the colon and breast. 6. SPS also can occur in patients with myasthenia gravis or thymoma. PATHOPHYSIOLOGY SPS is an autoimmune disorder caused by antibodies directed against glutamic acid decarboxylase (GAD) and amphiphysin. PROGNOSIS Patients develop progressive stiffness and rigidity of the trunk and spine. Immunomodulating therapies may help somewhat, but most patients still have significant disability. DIAGNOSIS Clinical Features 1. Progressive encephalomyelitis with rigidity is a rapidly progressive disorder associated with generalized stiffness, encephalopathy, myoclonus, and respiratory distress that is usually fatal within 6 to 16 weeks. 2. Typical SPS: a. Characterized by muscular rigidity and episodic spasms involving truncal and limb muscles in the second to sixth decades of life. b. Superimposed “ attacks ” of intense muscle spasms or contractions. c. The stiffness and muscles spasms usually lead to gait impairment with occasional falls. d. Patients may complain of dyspnea secondary to chest restriction due to stiffness in the thoracic muscles. e. Paroxysmal autonomic dysfunction characterized by transient hyperpyrexia, diaphoresis, tachypnea, tachycardia, hypertension, pupillary dilation, and occasional sudden death may accompany the attacks of muscle spasm. f. Approximately 10% of patients also have generalized seizures or myoclonus. g. Physical examination is remarkable for exaggerated lumber lordosis and paraspinal muscle hypertrophy secondary to continuous paraspinal muscle contraction. 3. Stiff limb syndrome is characterized by asymmetric rigidity and spasms in the distal extremities or face. Laboratory Features 1. Autoantibodies directed against the 64-kD GAD are evident in 60% of primary autoimmune cases of SPS. 2. Antibodies are directed against a 128-kD presynaptic protein, amphiphysin, are present in some patients with presumed paraneoplastic SPS. 3. The CSF is often abnormal in patients with SPS demonstrating increased immunoglobulin G (IgG) synthesis, oligoclonal bands, and anti-GAD antibodies. 4. Other autoantibodies and laboratory abnormalities associated with concomitant autoimmune disorders [e.g., Hashimoto thyroiditis, pernicious anemia, hypoparathyroidism, adrenal failure, myasthenia gravis, systemic lupus erythematosus (SLE), rheumatoid arthritis]. P.201 5. Serum CK levels may be slightly elevated. Electrophysiologic Findings 1. Sensory and motor conduction studies are normal. 2. EMG demonstrates normal-appearing MUAPs firing continuously. TREATMENT 1. Symptomatic therapies: a. I usually initiate symptomatic treatment with diazepam 2 mg b.i.d. working up to a dosage of 5 to 20 mg three to four times a day. b. Next I start oral baclofen 5 mg t.i.d. which is increased up to 20 mg q.i.d. c. Intrathecal baclofen 300 to 800 µg/d may be tried if other agents are not tolerated or are unsuccessful. d. Other symptomatic agents with purported benefit include: clonazepam, dantrium, methocarbamol, valproate, vigabatrin, gabapentin, and botulinum toxin injection. 2. Various forms of immunotherapy may be tried to treat the underlying autoimmune basis and have been found to be beneficial in small trials. a. I usually give a treatment trial of intravenous immunoglobulin (IVIG) 2 g/kg monthly for 3 months and, if this is effective, subsequently spread out the dosing interval or reduce the dosage tailored to patient responsiveness. b. Plasma exchange can also be performed but needs to be repeated (as does IVIG) and thus is not curative. c. A trial of prednisone 0.75 to 1.5 mg/kg/d for 2 weeks, then 0.75 to 1.5 mg/kg ever other day for 2 to 4 months is tried if IVIG is ineffective. If prednisone is beneficial, I taper the prednisone to the lowest dose that controls the symptoms. I do not use prednisone in patients with diabetes mellitus (DM). d. Other immunosuppressive agents (e.g., azathioprine, mycophenolate mofetil; Table 8-1) may be tried singly or in combination with prednisone as a steroid-sparing agent. TABLE 8-1. IMMUNOSUPPRESSIVE/IMMUNOMODULATORY THERAPIES COMMONLY USED IN NEUROMUSCULAR DISORDERS Therapy Route Dose Side Effects Monitor Prednisone p.o. 100 mg/d for 2 – 4 wk, then 100 mg every other day; single a.m. dose Hypertension, fluid and weight gain, hyperglycemia, hypokalemia, cataracts, gastric irritation, osteoporosis, infection, aseptic femoral necrosis Weight, blood pressure, serum glucose/potassium, cataract formation Methylprednisolone IV 1 g in 100 mL/normal saline over 1 – 2 h daily or every other day for 3 – 6 doses Arrhythmia, flushing, dysgeusia, anxiety, insomnia, fluid and weight gain, hyperglycemia, hypokalemia, infection Heart rate, blood pressure, serum glucose/potassium Azathioprine p.o. 2–3 mg/kg/d single a.m. dose Flu-like illness, hepatotoxicity, pancreatitis, leukopenia, macrocytosis, neoplasia, infection, teratogenicity Monthly CBC, liver enzymes Methotrexate p.o. 7.5 – 20 mg/wk; Hepatotoxicity, Monthly liver enzymes, single or divided doses; 1 d/wk dosing pulmonary fibrosis, infection, neoplasia, infertility, leukopenia, alopecia, gastric irritation, stomatitis, teratogenicity CBC; consider liver biopsy at 2 g accumulative dose IV/IM 20 – 50 mg weekly; 1 d/wk dosing Same as p.o. Same as p.o. Cyclophosphamide p.o. 1.5 – 2 mg/kg/d; single a.m. dose Bone marrow suppression, infertility, hemorrhagic cystitis, alopecia, infections, neoplasia, teratogenicity Monthly CBC, urinalysis IV 1 g/m 2 Same as p.o. (although more severe), and nausea/vomiting, alopecia Daily to weekly CBC, urinalysis Chlorambucil p.o. 4–6 mg/d single a.m. dose Bone marrow suppression, hepatotoxicity, neoplasia, infertility, teratogenicity, infection Monthly CBC, liver enzymes Cyclosporine p.o. 4 – 6 mg/kg/d split into two daily doses Nephrotoxicity, hypertension, infection, hepatotoxicity, hirsutism, tremor, gum hyperplasia, teratogenicity Blood pressure, monthly cyclosporine level, creatinine/BUN, liver enzymes Mycophenolate mofetil p.o. Adults (1 g b.i.d. to 1.5 g b.i.d.)Children (600 mg/m 2 /dose b.i.d.(no more than 1 g/d in patients withrenal failure) Bone marrow suppression, hypertension, tremor, diarrhea, nausea, vomiting, headache, sinusitis, confusion, amblyopia, cough, teratogenicity, infection, neoplasia CBCs are performed weekly for 1 mo, twice monthly for the second and third month, and then once a month for the first year Intravenous Immunoglobulin IV 2 g/kg over 2 – 5 d; then every 4 – 8 wk as needed Hypotension, arrhythmia, diaphoresis, flushing, nephrotoxicity, headache, aseptic meningitis, anaphylaxis, stroke Heart rate, blood pressure, creatinine/BUN p.o., by mouth; IV, intravenous; IM, intramuscular; b.i.d., twice a day; CBC, complete blood count; BUN, blood urea nitrogen. Modified from Amato AA, Barohn RJ. Idiopathic inflammatory myopathies. Neurol Clin 1997;15:615 – 648, with permission. Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition TETANUS Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND 1. Tetanus is a very serious and potentially life threatening medical condition arising from the in vivo production of a neurotoxin from the bacterium Clostridium tetani. 2. C. tetani produce tetanospasmin. 3. It is estimated that more than 1 million people per year demonstrate signs of clinical intoxication secondary to infections with C. tetani. About 150 cases of tetanus are noted each year in the United States by various governmental agencies. PATHOPHYSIOLOGY 1. The bacteria or their spores gain access to the patient typically through a minor wound. 2. In the central nervous system (CNS), tetanus toxin lyses the SNARE proteins necessary for the release of inhibitory neurotransmitters [glycine and γ-aminobutyric acid (GABA)]. P.202 P.203 3. The result is hyperexcitability of motor neurons leading to continuous motor unit firing, opisthotonus, and hyperreflexia. PROGNOSIS 1. The annual mortality rate due to this organism is variable depending upon the sophistication of emergent health care delivery and immunizations. 2. In Africa, the annual mortality rate is estimated at 28/100,000, while in Asia and Europe it is 15/100,000 and 0.5/100,00, respectively. 3. In the United States, the mortality due to tetanus intoxication is less than 0.1/100,000. 4. Worldwide, neonatal tetanus represents about 50% of the known cases with a mortality rate reaching 90%. DIAGNOSIS 1. The clinical presentation of tetanus is subdivided into four major categories: a. Local, b. Generalized, c. Cephalic, and d. Neonatal. 2. Most patients complain of a feeling of increased “ tightness ” of the muscles about the wound in the affected extremity. Pain may also be noted. 3. Both the pain and muscle stiffness can persist for months and remain localized with an eventual spontaneous dissipation. 4. Some patients develop trismus (difficulty opening the mouth secondary to masseter muscle contraction). 5. Progression to generalized tetanus with tonic contraction of either entire limbs or the whole body secondary to relatively mild noxious stimuli. The generalized whole-body muscle contraction, opisthitonus, consists of extreme spine extension, flexion and adduction of the arms, fist clenching, facial grimacing, and extension of the lower extremities. This generalized contraction may impair breathing. 6. Neonatal tetanus is usually the result of an infected umbilical stump. a. Several hours to days of feeding difficulty (poor suck), general irritability, and possibly less than normal mouth opening or generalized “ stiffness. ” b. Infants born to immunized mothers rarely have any difficulty with tetanus as the immunity is passively transferred from mother to infant. Once the massive whole-body contractions start, there is little doubt as to the diagnosis. TREATMENT 1. Patients with suspected tetanus intoxication should be hospitalized immediately and evaluated for existent or impending airway compromise. 2. Human tetanus immunoglobulin should be administered as well as adsorbed tetanus toxoid at a different site. 3. The antibiotic of choice is metronidazole (500 mg IV every 6 hours for 7 — 10 days). 4. If airway compromise is noted, there is a good chance that this situation will persist for some time and a tracheotomy should be considered. 5. Benzodiazepines should be administered in rather large dosages to control muscle contractions. If this is ineffective, therapeutic neuromuscular blockade is warranted in addition to the benzodiazepines to maintain somnolence. 6. If autonomic symptoms or signs develop, these should be treated immediately with appropriate medications. 7. Physical and occupational therapy are usually needed during the recovery period to regain strength, endurance, and function. [...]... represent the two extremes of disease manifestation 3 The tuberculoid form defines one end of the spectrum, in which the T H 1 cells predominate The T H 1 cells produce interleukin-2 (IL-2) and γ-interferon, which in turn lead to activation of macrophages 4 On the other extreme, the lepromatous form is dominated by T H 2 cells, which produce IL -4 , -5 , and -1 0, thereby down- regulating cell-mediated immunity... channels Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition IDIOPATHIC AUTONOMIC NEUROPATHY Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND 1 There is heterogeneity in the onset, the type of autonomic deficits, the presence or absence of somatic involvement, and the degree of recovery 2 Approximately 20% of patients have... modes of therapy Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition VASCULITIC NEUROPATHIES Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND 1 Vasculitis is a histologic diagnosis requiring transmural inflammation and necrosis of the blood vessel walls P.218 2 Vasculitic disorders can be classified on the basis of caliber... primarily myeloperoxidase or p-ANCA because of its perinuclear staining pattern These p-ANCA antibodies are present in as many as two thirds of patients 4 WG: Evaluation is remarkable for the presence of antineutrophil antibodies directed against proteinase-3 (c-ANCA) The specificity of c-ANCA for WG is 98% and the sensitivity is 95% The vasculitis is similar to that of PAN, affecting medium and small...Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition GUILLAIN—BARRÉ SYNDROME AND RELATED NEUROPATHIES Part of "8 - Motor Neuropathies and Peripheral Neuropathies" P.2 04 BACKGROUND 1 There are three major subtypes of Guillain—Barré syndrome (GBS): acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor-sensory axonal neuropathy... ©20 04 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition MILLER—FISHER SYNDROME Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND 1 In 1956, C Miller Fisher reported three patients with ataxia, areflexia, and ophthalmoplegia having a syndrome distinct from GBS 2 There is a 2 to 1 male predominance with a mean age of onset in the early 40 ′s... Lepromin Test induration) Bacterial Index 0 2 4 5–6 Morphologic Low (down to 0) Moderate High (up to 10) Cell-mediated immunity: intact; T H 1 > T H 2 lymphocytes; Cell-mediated immunity: unstable can Cell-mediated immunity: absent; T H 2 > T H 1 Cytokines expressed: IL-2, γ-IF range and switch from lymphocytes; cytokines intact to absent expressed: IL -4 , -5 , -1 0 Few localized and well Size, number, and... trials of patients with MFS 2 However, I treat patients with either IVIG 2 g/kg over 5 days or PE 250 mL/kg over 2 weeks similar to GBS Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND 1 CIDP is an immune-mediated... palsy) can receive IV penicillin G 250,000 U/kg/d in divided doses for 10 to 14 days or ceftriaxone 50 to 80 mg/kg/d IV for 2 to 4 weeks Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition HUMAN IMMUNODEFICIENCY VIRUS—ASSOCIATED DISTAL SYMMETRIC POLYNEUROPATHY Part of "8 - Motor Neuropathies and Peripheral Neuropathies" BACKGROUND Distal symmetric... cyclophosphamide at a dose of 1.0 to 2.0 mg/kg is a more potent suppressor of the immune system, but is associated with more adverse side effects (e.g., hemorrhagic cystitis) than IV pulses b I prefer monthly IV pulses of cyclophosphamide at a dose of 500 to 1,000 mg/m 2 of body surface area c Sodium 2-mercaptoethane sulfonate (Mesna) 20 mg/kg p.o every 3 to 4 hours for 12 to 24 hours each month on day of IV infusions . 1997;15:615 – 648 , with permission. Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition TETANUS Part of "8 - Motor Neuropathies. illness. Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition POSTPOLIOMYELITIS SYNDROME Part of "8 - Motor Neuropathies. section. Copyright ©20 04 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition STIFF PERSON/STIFF LIMB SYNDROME Part of "8 - Motor Neuropathies