By O. Fraser. Medical College of Wisconsin.

Respiratory weakness is rarely the presenting feature of ALS buy generic wellbutrin 300 mg on line, but becomes common with disease progression purchase wellbutrin 300mg. Patients initially experience exertional dys- pnea and sigh frequently when at rest. This continues on to dyspnea at rest, sleep apnea, morning headaches, and the inability to sleep supine. Nearly one third of ALS patients report urgent and obstructive micturition. Over time, muscles become atrophied and patients complain of fatigue. Signs As ALS affects both upper and lower motor neurons, most (80%) of patients show both upper and lower motor neuron signs. There is usually a combination of spasticity, hyperreflexia, and progressive muscle weakness and wasting. A small percentage of patients will only show lower motor neuron signs and symptoms. On the other hand, there are rare instances where patients only have upper motor neuron disease. There is currently debate as to whether this condition, called Primary Lateral Sclerosis (PLS), is a separate entity. The diagnostic procedures and treatments for PLS are currently identical to those for ALS. Pathogenesis Most cases of ALS (at least 80%) are sporadic. A smaller number are attributable to autosomal dominant familial ALS (FALS). The cause of sporadic ALS is currently unknown, although proposed etiologies include glutamate neurotox- icity, abnormal accumulation of neurofilaments, altered neurotrophism, and toxicity from oxygen radicals or environmental sources.

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For the ascending There are two enlargements of the cord: at the cervical pathways 300 mg wellbutrin visa, this means that sensory information from the level for the upper limb (seen at greater magnification in periphery is no longer available to the brain cheap 300mg wellbutrin with visa. On the motor Figure 2B), the roots of which will form the brachial side, all the motor commands cannot be transmitted to the plexus, and at the lumbosacral level for the lower limb, anterior horn cells, the final common pathway for the the roots of which form the lumbar and sacral plexuses. The person therefore is completely cut off The cord tapers at its ending, and this lowermost portion on the sensory side and loses all voluntary control, below is called the conus medullaris. Bowel and bladder control are also of L2 in the adult, inside the vertebral canal, are numerous lost. SPINAL CORD 3 CLINICAL ASPECT SPINAL CORD: CERVICAL REGION Because of its tenuous blood supply, the spinal cord is (PHOTOGRAPH) most vulnerable in the mid-thoracic portion. A dramatic drop in blood pressure, such as occurs with a cardiac This is a higher magnification photographic image of the arrest or excessive blood loss, may lead to an infarction cervical region of the spinal cord. The result can be just as severe as if roots are the motor/ventral roots, coming from the ventral the spinal cord was severed by a knife. The most serious horn of the spinal cord (discussed with Figure 4); a few consequence of this would be the loss of voluntary motor of the dorsal/sensory roots can be seen, which enter the control of the lower limbs, known as paraplegia. These roots exit the vertebral canal clinical picture will be understood once the sensory and and carry a sleeve of arachnoid-dura with them for a very motor tracts of the spinal cord have been explained (in short distance, as they head for the intervertebral spaces Section B). Surgeons who operate on the abdominal aorta, for The somewhat tortuous artery running down the mid- example, for aortic aneurysm, must make every effort to line of the cord is the anterior spinal artery. This artery, preserve the small branches coming off the aorta as these which is the major blood supply to the ventral portion of are critical for the vascular supply of the spinal cord. One the upper part of the cord, is formed by a branch from would not want the end result of an aneurysmal repair to each of the vertebral arteries (see Figure 58). The most vulnerable area of the spinal cord blood supply Embryologically, the spinal cord commences as a tube of is around the mid-thoracic level. In those segments that innervate the limbs important branch off the aorta that supplies this critical (muscles and skin), all the neurons reach maturity.

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The second rotation through an angle θ about the i axis (Fig effective 300 mg wellbutrin. The orthogonal transfor- mation matrix [T] resulting from the above rotations is given by cos cos cos sin sin sin    − sin cos sin sin cos cos   []T =  sin cos sin sin cos sin  (3 purchase 300 mg wellbutrin with visa. The position vectors of the contact points C (i = 1, 2) in the base ( i, j,kˆˆˆ ) are denoted by i r ˆ ˆ + ˆ (3. Furthermore, the unit normals to the surfaces of the moving and fixed body segments at the points of contacts must be colinear. Let nc (i = 1, 2) be the unit normals to the fixed surface, y = f(x, z), at the contact points, C (i i = 1, i 2), then 1  ∂rc   ∂rc  nˆ = i × i i = 12, (3. Similarly, following the i same procedure as outlined above nˆc′ (i = 1, 2), the unit outward normal to the moving surface, y′ = i g(x′, z′), at contact points, C (i i = 1, 2), and expressed in the x,y,zˆ ˆ ˆ system, can be written as      β ∂g ˆ ˆ ∂g ˆ nˆc′ =   ′ − ′ +   k′ (3. Colinearity of unit normals i at each contact point C (i i = 1, 2), requires that T = []T ˆ′ (3. Ligament and Contact Forces During its motion the moving body segment is subjected to the ligament forces, contact forces, and externally applied forces and moments (Fig. The contact forces and the ligament forces are the unknowns of the problem and the external forces and moments will be specified. These forces are discussed in some detail in the following paragraphs. The ligaments are modeled as nonlinear elastic springs. To be more specific, for the major ligaments of the knee joint, the following force-elongation relationship can be assumed: F = K (L – )2 for L > (3. The tensile force in the jth ligament is designated by F j.

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Patients have diffuse proximal weakness and bulbar symptoms with dysphagia and dysarthria safe 300 mg wellbutrin. Involvement of the extraocular muscles may result in diplopia and ptosis generic wellbutrin 300mg mastercard. Signs Proximally accentuated weakness with reduced or absent tendon reflexes. Autonomic signs consist of: Bradycardia Gastrointestinal symptoms: Nausea, constipation, diarrhea Hypohydrosis Hypotension Pupils dilated, blurred vision Urinary retention Clinical types – “Classic botulism” comes from ingestion of contaminated foods (home canned goods, garlic oil). Symptoms of oculobulbar weakness occur within 2–36 hours. Symptoms occur in a descending pattern, affecting upper limbs and lower limbs. Pupil dilation may be observed in half of the patients. Sympa- thetic and parasympathetic nerve transmission is also impaired. Intensive care may be necessary, and recovery is often prolonged but complete. Botulinum spores are ingested and proliferate in the gastrointestinal tract. Symptoms include weak crying, feeding difficulties, and weak limb muscles. Differential diagnosis: Other types of hypotonia (myopathy, GBS, familial MG, spinal muscular atrophy, poliomyelitis). Intravenous administration of recreational drugs can cause abscesses that lead to wound botulism. Prolonged jitter and increased blocking can be observed in SFEMG. Suspected food should be tested for the bacteria and toxin. Diphtheric paralysis Differential diagnosis GBS Miller Fisher syndrome MG Tick Paralysis Descending symptoms are the hallmark, as opposed to ascending symptoms in GBS Supportive care Therapy Antitoxin administration is controversial Guanidine, 3,4-aminopyridine (Drugs to facilitate the presynaptic release).