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How to Diagnose and Treat Neuromuscular Diseases: “The Weak, Trembling Horse”

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How to Diagnose and Treat Neuromuscular Diseases: “The Weak, Trembling Horse” MEDICINE—NEUROLOGY How to Diagnose and Treat Neuromuscular Diseases: “The Weak, Trembling Horse” Joan L. Norton, VMD; and Amy L. Johnson, DVM, Diplomate ACVIM Authors’ address: Department of Clinical Studies, New Bolton Center, University of Pennsylvania, Kennett Square, Pennsylvania 19348; e-mail: [email protected] (Norton). © 2009 AAEP. 1. Introduction botulinum, an anaerobic, spore-forming rod. Botu- Disease states affecting the lower motor neuron lism spores are found in soil throughout the United (LMN), neuromuscular junction, or muscles can result States. There have been eight toxin types identi- 1 in vague clinical signs of weakness and trembling that fied (A, B, C1, C2, D, E, F, and G). More than 85% make these diseases difficult to distinguish from one of cases seen in North America are caused by toxin another as well as from non-neuromuscular problems B.2 Infection can occur in one of three ways: in- such as electrolyte disturbances, colic, or pain. Early gestion of the preformed toxin, ingestion of spores recognition of neuromuscular disease is important with subsequent elaboration of toxin in the gastro- in rapid diagnosis and early, appropriate treatment. intestinal tract (Shaker Foal Syndrome), or contam- Clinical signs in acute cases often include trembling, ination of a wound with spores leading to production tachycardia, unwillingness to stand still, an abnor- and absorption of the toxin. Equine cases usually mal stance (elephant on a ball), repeated attempts to occur sporadically, whereas bovine cases can be seen lie down, and prolonged periods of recumbency. as herd outbreaks.3 There are several disease processes that have been The toxin acts at the presynaptic nerve terminal recognized in the horse that result in these signs to block release of acetylcholine into the synaptic either acutely or chronically. This summary de- cleft. The toxin binds irreversibly to receptors on scribes these diseases and presents a stepwise pro- the presynaptic terminal and is internalized in a cess that practitioners can use to differentiate vesicle. Because the biding is irreversible, neuro- the diseases from each other as well as from more muscular function only improves when nerve end- common causes of weakness and trembling. In ad- ings have been regenerated. dition, this summary reviews diagnostic and thera- Clinical signs are the result of muscle weakness peutic options. that may progress to diffuse flaccid paralysis. These signs can occur anywhere from 12 h to 10 days 2. Botulism after intoxication or infection. The most classic Botulism is one of the more common LMN diseases clinical signs associated with botulism in horses are seen in the horse. The signs seen are the result of poor tongue tone and dysphagia. Early signs in- a neurotoxin produced by the bacterium Clostridium clude generalized muscle weakness, gait abnormal- NOTES AAEP PROCEEDINGS ր Vol. 55 ր 2009 167 MEDICINE—NEUROLOGY ities (stiff/shuffling), decreased ability to eat, and Aminoglycosides (gentamicin and amikacin) and increased episodes of recumbency. These horses polymixin B should be avoided.6 Lidocaine will have normal mentation because the central nervous also affect neuromuscular transmission and should system (CNS) is not affected. Cranial nerves re- not be used in these cases.7 Although these horses main intact though there can be a decrease in lid have difficulty standing for prolonged periods, resist tone and pupillary light reflexes. the urge to place them in a sling. This may lead to Diagnosis of botulism is usually presumptive and struggling, exhaustion, and increased mortality. based on clinical signs, as well as vaccination his- Mechanical ventilation may be required in the most tory. A “grain test” can be performed. This in- severe cases and is feasible in foals but is not usually volves feeding 8 oz of grain in a shallow tub. A possible in the adult horse. normal horse should be able to finish the grain in Ͻ2 Outcome and duration of recovery are dependent min.4 Hematology and serum chemistry may re- on the amount of toxin that is initially present in the flect dehydration and horses with prolonged diffi- circulation. Prognosis is difficult to determine at culty standing can have muscle enzyme elevations. the onset of clinical signs, and success can be depen- Cerebrospinal fluid (CSF) is normal. Definitive di- dent on the secondary complications of recumbency agnosis is made by the demonstration of toxin in the such as pneumonia and decubital ulceration. A gastrointestinal (GI) contents, serum, or wounds. highly effective vaccine is available in North Amer- The most sensitive test is a mouse bioassay where ica but only protects against type B toxin. Horses serum of a suspected horse is injected into a mouse, in endemic areas should be vaccinated. which is observed for signs of botulism. Mice that have been pretreated with antiserum are also in- 3. Equine Motor Neuron Disease jected and should be protected if the toxin is present. Unfortunately, horses are sensitive to low levels of Equine motor neuron disease (EMND) is a more toxin that may not produce disease in these mice; recently reported disease of horses. It is associated therefore, many cases of equine botulism are not de- with decreased serum vitamin E levels, and the dis- finitively diagnosed. Enzyme-linked immunosorbent ease can be reproduced by holding horses off fresh assay (ELISA) and polymerase chain reaction (PCR) grass pasture for months to years and feeding low tests have been developed but are not commercially vitamin E diets. Small intestinal malabsorptive available. diseases could also lead to low levels of vitamin E The most important treatment in the manage- and other antioxidants. Because histopathologic ment of a botulism case is the administration of changes are similar to those of amyotrophic lateral antitoxin. The antitoxin binds and neutralizes the sclerosis (ALS), oxidative stress likely is involved in circulating toxin. Timing is important because the the observed neuronal injury and denervation. outcome is favorable in 70% of cases when the anti- These changes are especially seen in type I myofi- toxin is administered while the horse is still stand- bers, which are more oxidatively active than type II ing.5 The prognosis decreases significantly once fibers. the horse becomes recumbent and is highly fatal if Clinical signs are reflective of the skeletal muscle the antitoxin is not administered at all except in denervation and include generalized weakness and very mild cases. A polyvalent antitoxin is available muscle atrophy. The onset can be insidious or from the University of Pennsylvania, New Bolton acute. It is not uncommon for these cases to Center. This form of antitoxin is available in present for chronic weight loss because of the 500-ml units, and one unit is usually sufficient for a chronic nature of the muscle atrophy. As the pos- 500-kg adult horse. A monovalent antitoxin is tural (antigravity) muscles are predominately af- available from Veterinary Diagnostics Inc. in fected, trembling and increased periods of Templeton, CA. It is important to anticipate a pro- recumbency are often seen. These horses also gression in clinical signs for 12–24 h after adminis- stand in a characteristic position of an “elephant on tration of the antitoxin because it can only bind a ball” with all four feet placed close together to freely circulating toxin. The toxin that has al- better distribute weight over all four weak limbs. ready entered nerve terminals will still bind with These animals also frequently shift their weight and receptors.5 are more comfortable walking than stand- The remainder of treatment in these cases is ing. Thus, they are often perceived to be anxious or mainly supportive. The dysphagia commonly seen in pain. Head carriage in these cases is often low, will necessitate nutritional support, either enterally and there can be wasting of the neck and shoulder or parenterally. Ileus is another sequelae that may musculature. Conversely the tail head in these require withholding enteral feeding. Horses that cases appears elevated because of the atrophy of the are dysphagic may aspirate. This tendency, com- coccygeal muscles. Mentation and cranial nerves bined with prolonged recumbency, predisposes these are unaffected, and no proprioceptive ataxia is seen cases to aspiration pneumonia, and antimicrobial in these cases. In some instances, fundic examina- therapy may be necessary. It is important when tion will show deposition of brown-black or yellow- treating these cases to avoid the use of pharmaceu- brown lipofuscin pigment at the tapetal–non-tapetal ticals that potentiate the neuromuscular blockade. junction.5 168 2009 ր Vol. 55 ր AAEP PROCEEDINGS MEDICINE—NEUROLOGY Diagnosis is based on clinical signs and history of is known to be HYPP homo- or heterozygous or if poor access to green forage. The most consistent HYPP status is unknown. biochemical abnormality in these cases is hypovita- minosis E. Increases in muscle enzyme activity may 6. Other Myopathies be present in acute cases but may also be normal in Two additional myopathies, polysaccharide storage more chronic states. There are no typical changes myopathy (PSSM) and recurrent exertional rhabdo- seen in CSF. Muscle or nerve biopsies will show myolysis (RER), warrant mention because these denervation. These changes along with appropri- cases can present with similar clinical signs. Like ate clinical signs are needed for a definitive diagno- HYPP, these myopathies are often associated with sis of EMND. The muscle biopsy is the most recent exercise. RER is more often described in common invasive diagnostic. The sacrocaudalis Thoroughbreds, whereas PSSM is associated with dorsalis medialis, the tailhead muscle, is predomi- Quarter Horses and Draft breeds. The exact mech- nantly type I fibers and highly sensitive to denerva- anism that causes RER has not been identified, but tion. This sample can be easily obtained with it is thought to be a defect in calcium regulation.
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