First described as a specific, separate disease in 1973, GSD myelopathy has been around a long time, but previously not as well understood or distinguished from other pathologies. It gets its common name from the fact that German Shepherd Dogs are found relatively often, compared to much more uncommonly in a few other large breeds. As a matter of fact, the peculiar syndrome seen in our breed is also seen only (though rarely) in the Belgian Shepherd and the Old English Sheepdog, and Dr. R. M. Clemmons, neurology professor at University of Florida's veterinary school, feels what is seen in other breeds may well be a different disorder. Those other degenerative myelopathies are probably not caused by the same immune-system-related deficiency as we have in the GSD.

Diagnosis is by the process of elimination, since regular neurological tests aren't definitive, except for the presence of a brain fluid protein in the lumbar area of the cord. Your best bet would be to consult an experienced breeder who's been through it with his own dog or dogs, and use the local vet for confirmation and medication. He will see what you have: the dog atrophies in the haunches and croup. Autopsy will not help you or your dog, but would find the obvious demyelination (loss of the insulating sheath) of the spinal cord, destruction of some large axons (nerve cells leading from the cord to smaller branch nerves), and abnormal cells (or certain cells in abnormal locations). Similar signs may be seen in the brain's white matter, and plasma cell infiltrates in the kidneys and intestines give further evidence of the immune system failure at the root of this disease.

Since GSD myelopathy is so similar to multiple sclerosis, perhaps it would be worth trying the nutritional approaches used by some who treat that human disorder. Wheat germ oil contains octocosanol, a fatty or waxy high molecular-weight alcohol also given to patients with encephalitis and cerebral palsy, and also contains linoleic acid (as do several vegetable oils). But one fatty acid, docosahexanoic acid, is not at all abundant in normal diets, yet seems to be the first one depleted from the nerves' myelin sheaths in such disorders as MS. In many diets for MS patients, foods such as sardines and mackerel are emphasized, because they are among the very few foods that can supply this compound. Of course, vitamin B complex high in inositol, B6, B12, and choline is also highly recommended as an adjunct to vitamin E for nerve-muscle disorders. Since only a percentage of patients with MS or DM respond satisfactorily to a vitamin-only approach, the concomitant use of drugs and exercise should always be prescribed. As of this writing, there were three FDA-approved drugs for MS that not only help manage symptoms but also appear to "impact disease course" in relapsing MS, according to the National Multiple Sclerosis Society. They are Copaxone (Teva Marion), Avonex (Biogen), and Zanaflex (Athena Neurosciences). Your vet would have to decide whether or not he wanted to "go off-label", since these have not (yet?) been approved for dogs.

For several years, there was no generally accepted treatment, but as breeders became aware of the benefits of high vitamin E dosages, the veterinary profession began to see its efficacy and the relation to the immune system. When I first encountered it, I got an extra one or two years useful life out of one of my dogs by giving him 800 units (IU) a day, plus vitamin C for its synergistic effect. Years later it was discovered that 2000 IU of vitamin E daily, 500 mg of vitamin C twice a day, and a high-strength vitamin B complex twice a day is the best dosage. Vitamin E is an important nutrient with a number of physiologic and pharmacological effects. As an antioxidant it helps reduce oxidation of fats and increases the production of HDL cholesterol. At higher doses it decreases production of prostaglandins and has anti-inflammatory action. There is no known side-effects to vitamin E at levels less than 4000-6000 IU per day (except in cats, where levels above 100 IU/day can create hepatolipidosis. In DM, low serum and tissue concentrations of vitamin E have been observed. I recommend that vitamin E be given to all German Shepherd Dogs. For GSDs less than 2 years of age, give 400 IU of vitamin E daily. For older GSDs, give 800 IU of vitamin E daily. If your dog develops DM, then the dose of vitamin E should be increased to 2000 IU daily. Dr. Clemmons recommends the vitamin E be dropped temporarily to about 100 IU if the dog has to be given aspirin for any reason during the treatment, and recommends that daily DEC (diethylcarbamazine) replace the monthly heartworm medications ivermectin (Heartgard, Heartgard Plus, Ivomec) and Interceptor because these increase immune responsiveness; also use the DEC in place of styrid caracide or Filaribits. Personally, I would simply stop all use of heartworm medication, because the dog with DM probably isn't going to last as long as it takes for a case of heartworm to become life threatening, anyway. Flea control should be limited to Precor™ for the house, and carbamates or pyrethrin/pyrethrum on the dog.

Chemical-pharmacological treatment has largely been via the use of aminocaproic acid, something my friend Wayne Riser (founder of OFA) told me about many years ago. More recently, acetylcysteine three times a day has found acceptance. It now appears the best treatment is a combination of all three approaches, along with exercise. Alternate day dosage with a steroid such as prednisone, plus acetylcysteine, added to the aminocaproic acid and vitamin formula, is enough to keep the dog owner very busy and tied to the home, but it offers the best chance at reducing progression, thus prolonging life considerably more than in the past. The drugs should be given in liquid form, mixed with a palatable solution such as chicken broth. There are generic varieties available. Medication helps up to 80% of afflicted dogs, especially if started early. Hydergine, a prescription drug derived from ergot fungus, is being researched, since it seems to promote nerve regeneration. For dogs with advanced DM, Dr. Clemmons suggests trying 5 mg three times a day for at least three months.

I mentioned exercise, and this is the third of the four-part fight against the disease. Every other day, the dog should be given at least 30 minutes of aerobic activity such as vigorous walking or swimming; if you start late, build up to the strenuous level gradually; if your dog can't do the most, do less, but make it regular and stretch the limits where you can. The alternating days of relaxation are important for repair and renewal. If the dog acts sore, give him the analgesic prescribed by your vet (after he has "read up" on the interactions of medications in regard to GSD myelopathy. The fourth part is stress reduction. The vitamin C, that anti-stress vitamin, is there for a good reason, but take steps to avoid stressful situations, including surgery; if the dog needs surgery, make sure he gets the acetylcysteine as well.

Dogs with GSD myelopathy often develop lick granulomas, which are ulcerations or (if you are lucky) callous-like reactions of the skin to extremely frequent licking, sometimes chewing, at the location of a supposed itch. It is probably a case of the limb feeling as if it has "fallen asleep", to put it into terms familiar to human experience. The tingling sensation caused by incomplete and erroneous signals by the nerves serving that place is much like the irritation caused by an ant bite, or hairs out of place, or anything in between. In trying to lick it away, the dog actually softens and wears away the hair and skin. The best I could do for my dog was prick the vitamin E capsule, squeeze out a little for topical application to the granuloma, pop the rest into his mouth, and then give him some distraction such as go for a walk. These ulcers on the feet or elsewhere don't easily respond, but the battle must be waged if the dog's life and comfort are to be prolonged. The immune system insufficiency may be temporary, though it is more often just "managed" or covered up. It may limit itself to just one or two symptoms such as the biting/nibbling/licking.

The approach to treatment of GSD Myelopathy that is proposed by Dr. Clemmons is called "integrative treatment". It combines conventional pharmaceutical treatment with "alternative medicine" or "supportive therapy". Paraphrasing some of his comments might be helpful here. Conventional drug therapy (medicines) has been of little lasting help to patients with DM. However, the combination of exercise, vitamins, and certain drugs has delayed the progression of DM in many dogs. Treatment has been directed at suppression of symptoms, and since the cause of this autoimmune disease is not known, little has been done in the way of finding out how to prevent it. There are possibly genetic, environmental and toxic factors involved. Saying that Degenerative Myelopathy is an autoimmune disease means the animal's immune system attacks its own cells; in this case, the central nervous system. The myelin insulation sheath around the nerves and axons (fibers) is gradually destroyed. It's worst in the thoraco-lumbar area of the spinal cord, but can also affect the brain stem and other nerve tissue.

Integrative or supportive treatment of German Shepherd Dog Myelopathy, as promoted at the University of Florida vet school, suggests the use of dietary alternatives and supplements to combat the immune system, and is derived from an approach to treating Multiple Sclerosis. You probably know at least one person with MS, and can recognize the similarity in symptoms. Besides the vitamins E and C, the drugs, and the exercise mentioned above, avoidance of toxins such as is found in pesticides and lawn chemicals, and perhaps in some processed foods, is possibly helpful. If you cannot manage "home cooking" with its involved recipes, supplement dog food that is as natural as possible with recommended aids. Soybean curd (tofu) protein contains flavonoids; raw garlic (action is lost when is cooked or dried) is anti-bacterial and anti-fungal, and may benefit the dog by reducing immune challenge. Ginger is also anti-inflammatory and with garlic, can replace aspirin and other NSAIDs. Adding soy concentrate, soy lecithin, and beta-carotene and other vitamins to commercial food is recommended. Use "stress formula" B-complex containing 100 mg of most of the B vitamin components; or use yeast as a good source of these B-complex vitamins, trace minerals, and some protein. It is relatively inexpensive; try half a tablespoon mixed in each meal. If your dog gets flatulence from this, as most do, use a discount or mail-order vitamin B complex pill instead.

Antioxidant vitamins E and C are synergistic; i.e., they work together better than they do separately. While dogs produce vitamin C, those with DM may need more than they can manufacture. In excess, it also can cause flatulence. I normally recommend not supplementing with vitamin C for longer than a month or two at a time, but in the case of a permanent, chronic disorder like DM, it's better to continue usage. Tolerance in the intestines may be for as much as 3000 mg per day, but up to 1000 mg twice a day should be enough unless it causes diarrhea. Selenium also is synergistic, helping vitamin E to be more effective. It can be toxic to medium-size dogs if given at more than 200 µg (micrograms) of selenium per day. I take 200 µg whenever I feel I'm not getting much western grain (good source of selenium) in my daily diet (which is rarely, such as when I’m out of the country for an extended time), and I weigh twice as much as my largest German Shepherd Dog.

Clemmons says that "Omega-3 fatty acids such as EPA (eicosapentanoic acid) and DHA (docosahexanoic acid) are the constituents of fish oils that act as anti-inflammatory agents and may be worth trying if your dog has an autoimmune disorder or arthritis." Fortunate is the owner who can give an afflicted dog a couple of cooked sardines or a small piece of salmon as a daily, natural source of such fatty acids. A 1000-mg fish oil capsule, tablespoon of ground flax seeds, or flaxseed or wheat germ oil supplement can do about the same thing. If you are really "into" the health-food store shopping, 500 mg twice a day of GLA (gammalinolenic acid), a fatty acid found in evening primrose and black currant oils is an alternative anti-inflammatory without the side effects of most anti-inflammatory drugs. All of the above should be considered as optional adjuncts to conventional treatment with the drugs, vitamins, and exercise, not replacements for them.

Disorders with similar symptoms: Cauda equina syndrome, giant axonal neuropathy, and tumors may also mimic DM. Many cases of spinal and related nerve damage are due to sudden trauma, but some can result from encroachment of bone or tumors into the space occupied by the cord. Since nervous tissue generally does not regenerate, such conditions result in partial or complete paralysis. Symptoms similar to myelopathy may be brought on by a nerve cell degeneration normally associated with age. However, with the latter disorder, the rapidly progressive nature of GSD myelopathy is not seen. Neoplasms also cause the dog to display symptoms similar to those of GSD myelopathy. These tumors on the spinal cord, neuroepitheliomas, have a special predilection for German Shepherd Dogs from six months to maturity at three years of age.

If you have lost a dog to this neurological disorder, I offer my condolences in the form of a poem:

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Postscript:

Since this article was first published, a case came to my attention that represents one extreme of treatment/longevity in DM’s progression, the other extreme being a need for euthanasia within a couple months of first symptoms. This correspondent had a large number of health problems with her GSD and elected to wage a vigorous fight to prolong the dog’s life, ease his discomforts, and help him through the inconvenience and confusion brought on by DM.

I have read articles from you on DM and I have a question. We like your book and know that you are a true German Shepherd person. We have a GSD who has DM. I must tell you that he is the most wonderful dog in the world. He has had many health problems and has NEVER been cross. Even at the worst times he would act like a clown to make US happy. He has never as much as lifted a lip at anyone, all of his doctors have commented on how good he is. Even now we believe he still has the look of eagles.

His birthdate: 7/7/89 (This was written in May of 2001). Previous problems: Umbilical hernia, a bout of colitis, strange crystals in his eyes that do not affect vision but we were told by the eye specialist that she has only seen in GSD's. [Fred’s note: probably corneal dystrophy… see the book.]

Also, in November of 1989 he was sent to Dr. Manely U. of Wisconsin showing extreme rear angulation and pano. Walking on hocks. Sores and calluses evident. Was told that if he did not come up soon we may need to consider euthanasia. He did come up but remained what we consider "over-angulated." [Fred’s note: actually bragged about by many GSDCA-AKC American Shepherd breeders and owners!]

At the same time was referred to a dental specialist because the dog has an overshot jaw. The worst our vet had ever seen. The bottom canines were going to pierce the sinus cavity. He sent us to Dr. Scheels. The dental specialist took impressions and manufactured an orthodontic appliance. The appliance did not have to be used because when the upper canines were pulled, continued growth spread the bottom canines out. The case was used in an unpublished university study.

About 2.5 years old he began gran mal cluster seizures (epilepsy). These continued until about 6 years ago. About 4 or 5 years ago he began to move differently in the rear. Was diagnosed with DM about 3 years ago. We have had him on Winstrol™ [anabolic steroids] for the last 1.5 years. At one time I weaned him off of that drug, fearing side effects but he got very weak so we put him back on. He has been on Vitamins C, E, and B Complex for the last 8 years. He has had regular acupuncture treatments for about 1.5 years, and up until this last month they seemed to help him. Now, we don't notice any difference from the treatments. He has two vets, one who is his acupuncture vet and the other is his regular vet — both are wonderful.

He is fed a good quality kibble with raw meat, grated vegetables along with flaxseed oil and other extras. Ever since I started this about 6 years ago, the colitis disappeared and so did the seizures. I believe it was a combination of the right dose of drugs and diet. Since his seizures had stopped, we waited a year and then gradually lowered his dosage of the drugs. He has been on such a low dose of Phenobarbital and potassium bromide for the last 5 years that his vet thought we could stop but the memory of those horrible seizures kept us from stopping completely.

If we had to do it all over again we probably would. This dog is amazing. We love him very much. We are considering euthanasia soon because watching him is so painful to us. He has been tripping over his legs for over a year. His leg gets hooked over the other often now. Usually he will take a few "drunken" steps and then lay down BUT not always. He still follows me around the backyard or tries to. He lost control of his bowels about a year ago. Sometimes he makes it outside but now very often when he strains to get up it just starts dropping out of him. He is licking his front feet now; I believe he may feel tingling. [Fred’s note: one of the signs of an immune system disorder.] His mind says yes but the body won't cooperate. This dog has heart like I've never seen. When do you know it's time? [Fred’s note: my own cut-off date is when a dog can no longer get up and squat to defecate. Everyone has to decide on his own deadline, but you must have a plan and stick to it, or the event will be even harder on you.]

He is from good breeding: AKC Ch. Woodside Nestle Quik v. Merwestyn and Covy Tucker Hill's Marsaycon. [Fred’s note: while the collection of related and unrelated disorders may not be seen in German lines, DM certainly is not limited to American lines. It is widespread throughout the breed.] One undescended testicle.
No big deal of course. He couldn't be shown anyway. He was neutered at about 1 yr. old.He was "hell on wheels" as a puppy. He was born with spirit and brains. He breezed through training and the Canine Good Citizenship test, worked as a pet therapy dog for a short time before his health problems stopped us, failed the Temperament Test because he did not show aggression to the "weird stranger" at the end of the test. The tester told us that he performed perfectly until then, in fact, if he were to make a tape of how a dog should react to all the other parts, he could have used Bosco. I was and am very proud of him. He is so kind. When we brought home a stray female with 3-day old pups to foster about 4 years ago, he "mothered" them more then we did. He let the plumbers into the house while I was in the backyard one day and when I came in he had an assortment of his toys lined up for them. The guys laughed and said, "Lady, you have the friendliest German Shepherd we have ever seen!" - I asked them not to tell anybody.

I made one promise to him years ago. I would never let him suffer. We need to know, is he? How do you know when it's time? Can they still have that look? Is it right to put him down? I know these are hard questions but I need to know from someone familiar with this disease. We believe that if he is in pain he won't let us know. Yes, when he moves he looks pathetic. He has a lot of muscle wasting. Our own family said they couldn’t bear to watch him walk anymore. But he would still chase after a ball if we let him; he might be too weak or tired to bring it back, but I know he'll go out for it. He "runs" to his food dish at breakfast and supper. And that look. It's as if he's saying "What?" He still talks back to us and sleeps next to our bed. He now has no rules and that seems to his liking. This is so hard I can't stand it. A vet once told us that we’d know it's time when he loses interest in food and life. I'm telling you, that won't happen with this guy! Traveling is getting pretty hard for him."

T.

I hope readers can empathize with the individual, and the many like her, who have suffered along with their companions, from the ravages of this disease. I also hope she is able to find a replacement with better health and genetics.

Fred

Here is another sentiment for those of you who are still struggling with the problem: