Treatment
There is no treatment - however we started on a regime of supplements and diet change and Taj lived way beyond the median survival of Danes studied in one of the academic papers.
Supplements
Our specialist vet recommended the L-carnitine (an amino acid) and Coenzyme Q10 (as an antioxidant) supplements.
The doses we gave Taj are L-carnitine 3 mLs (6g) daily plus Coenzyme Q10 10 mg/kg daily (i.e. 600 mg for Taj).
I sourced the L-carnitine as a paste for horses and dogs from my local grain and feed store, but this is also available on the internet. I sourced the Coenzyme Q10 from an internet search (online health supplements). The Coenzyme Q10 is the most expensive product and it is worth shopping around and buying in bulk and Australian internet suppliers are not always the cheapest!
Diet
I think this made a real difference. We changed Taj's diet to BARF (bones and raw food) based on a book called 'Feed Your Dog a Bone' by an Australian vet Dr Ian Billinghurst who proposes a natural, uncooked diet for dogs. I strongly recommend you buy the book, it's cheap.
We were able to find a local butcher who will order in chicken carcasses, lamb offcuts and offal - we supply the vegies - then he minces the 20 kg lot in his commercial mincer. To this mix we add the supplements and we then package into 1 kg containers and freeze. As well we fed 50% raw bones.
You can get vitamins from your supermarket or health food shop. Health food shops sell Kelp powder and bulk Brewers Yeast.
Homeopathy
The 'no harm principle' - if it can't do any harm then give it a go, although Homeopathy is lunatic fringe stuff to many scientists and standard medicos!
Jan’s sister in New Zealand is a homeopath and gave Jan a homeopathic remedy to try on the understanding that this would not be a magic cure but could palliate the symptoms a little. Remedies can be sourced from homeopathic supplies shop.
Conium 30c. Give the dog one dose three times first day, then night and morning day two, then if no change (even in other areas apart from muscles) give the dog one more dose at night for another 5 days. Leave it a week and see what happens, then you can give one dose a day for another 5 days.
Supplements
Our specialist vet recommended the L-carnitine (an amino acid) and Coenzyme Q10 (as an antioxidant) supplements.
The doses we gave Taj are L-carnitine 3 mLs (6g) daily plus Coenzyme Q10 10 mg/kg daily (i.e. 600 mg for Taj).
I sourced the L-carnitine as a paste for horses and dogs from my local grain and feed store, but this is also available on the internet. I sourced the Coenzyme Q10 from an internet search (online health supplements). The Coenzyme Q10 is the most expensive product and it is worth shopping around and buying in bulk and Australian internet suppliers are not always the cheapest!
Diet
I think this made a real difference. We changed Taj's diet to BARF (bones and raw food) based on a book called 'Feed Your Dog a Bone' by an Australian vet Dr Ian Billinghurst who proposes a natural, uncooked diet for dogs. I strongly recommend you buy the book, it's cheap.
We were able to find a local butcher who will order in chicken carcasses, lamb offcuts and offal - we supply the vegies - then he minces the 20 kg lot in his commercial mincer. To this mix we add the supplements and we then package into 1 kg containers and freeze. As well we fed 50% raw bones.
You can get vitamins from your supermarket or health food shop. Health food shops sell Kelp powder and bulk Brewers Yeast.
Homeopathy
The 'no harm principle' - if it can't do any harm then give it a go, although Homeopathy is lunatic fringe stuff to many scientists and standard medicos!
Jan’s sister in New Zealand is a homeopath and gave Jan a homeopathic remedy to try on the understanding that this would not be a magic cure but could palliate the symptoms a little. Remedies can be sourced from homeopathic supplies shop.
Conium 30c. Give the dog one dose three times first day, then night and morning day two, then if no change (even in other areas apart from muscles) give the dog one more dose at night for another 5 days. Leave it a week and see what happens, then you can give one dose a day for another 5 days.
FAQs
Could any Great Dane be affected?
To our knowledge, IMGDhas only been reported in Fawns and Brindles.
Symptoms
Reported symptoms for IMGD include progressive muscle wasting, exercise intolerance, general body tremors and collapse.
In Taj's case, he has splayed front paws (outwardly facing), severe exercise intolerance which is probably connected to his severe heat stress, wobbly hind leg movements, inability to jump and at any speed over a walk he bunny hops i.e. his hind legs move together.
See videos.
Treatment(s)
There are no (medical) treatments. See the supplements and feeding regime for Taj that allowed him to live beyond dogs studied.
Also see info about humans:
http://home.goulburn.net.au/~shack/medications.htm
Progression of the disease
Dr Jim Anderson (Faculty of Veterinary Medicine, University of Glasgow) has a special interest in IMGD and responded to us about a study he was doing on IMGD in Great Danes:
"My impression is that dogs that present severely affected are euthanased after weeks/couple of months whilst those that present with a a relatively mild condition continue as they are. Some of the patients are still about having a reasonable quality of life many months or a number of years after diagnosis. Of the dogs we have follow up on about 25% are still with us. My guess this data is skewed towards the severely affected animals as they are most likely to have been through a diagnostic procedure. I suspect that there is quite a range in the severity of the clinical signs.
We do not have any really long term follow up but that probably reflects the nature of the study."
Read the precis and abstract of Dr Anderson's paper (May 2006) on this disease
Clinical definition of Central Core Myopathy
In humans (and other mammals) a variety of progressive, degenerative ‘muscle wasting’ disorders are collectively termed muscular dystrophy. The basis of each form of muscular dystrophy can be different – some involve neurological defects, others result from structural deficiencies in protein components of muscle tissue, and some are the consequence of metabolic disorders. In all cases of muscular dystrophy, some contribution (either great or small) is genetic and hence these disorders are often familial. In addition, a very few cases can be the result of spontaneous or de novo mutations in the genes that underlie the manifestations of the diseases.
Environmental factors (diet, exercise, exposure to certain chemicals) can also be important in influencing how genetic variations or mutations can result in disease severity.
People and dogs (and other mammals) have in common many diseases which have a genetic basis – our DNA and genes are very similar! Over the past two decades, the genetic defects responsible for a variety of human disorders have been first identified in animals exhibiting similar clinical features. In Taj’s case it is the other way around - Central Core Disease (CCD) in humans has been well studied and the gene responsible (RYR1 ) has been identified but CCM or IMDG in dogs has only recently been recognised.
In humans, mutations in a gene called RYR1 can result in at least three clinically-defined syndromes:
1. Malignant Hyperthermia (MH), seen as heat/exercise or anaesthetic intolerance
2. A form of severe skeletal malformation (properly called Spondylocostal Dystosis)
3. Central Core Disease (CCD) a form of muscular dystrophy.
The RYR1 gene produces a large protein involved in regulation of the flow of calcium in and out of muscle cells.
Diagnosis process
In Taj's case, diagnosis was confirmed after X-rays ruled out any skeletal problem such as hip dysplasia. He then went on to have a nerve and muscle biopsy which were sent off to the veterinary pathologist for analysis.
Under general anaesthetic, a small piece of muscle (and nerve) was cut out of his leg and sent to the veterinary pathologist. The pathologist stained the muscle tissue sample and then looked at the muscle cells under a microscope. They look for a dark stain which shows a core in the muscle cells. Hence the name of the disease – Central Core Myopathy.
It is possible that there has been misdiagnosis of other Great Danes - 'Wobblers' seems to be a disease that a number of Great Danes are diagnosed within Australia. While some symptoms may display in some similar ways, this is a completely separate disease.
Read the Case Study about Taj our vets have had published:
"Inherited myopathy in a Great Dane". SE Davies, DR Davies, RB Richards and WJ Bruce. Australian Veterinary Journal 2008;86:43–45.
Free to download from link: doi: 10.1111/j.1751-0813.2007.00202.x
Taj's diagnosis from the Veterinary Pathologist
Gross Pathology -Two splinted biopsies, one of muscle and the other of nerve.
Histopathology - The muscle biopsy contains several areas where there are numerous enlarged fibres with central clearing of myofilaments. The clear zones often have central nuclei and can be seen, in LS, to extend for short distances along the fibre. There are infrequent fibres with peripheral loss of myofilaments. Sub-sarcolemmal nuclei are enlarged adjacent to areas of myofilment loss, but elsewhere are normal. While most of the affected fibres appear to have enlarged diameters, there are occasional atrophic fibres in relatively unaffected bundles. There is an equivocal increase in perimysial connective tissue, but no additional cells are present in the muscle sections. The sections of peripheral nerve show normal morphology.
Diagnosis - Focal myofibrillar lysis (muscular dystrophy)
Comments - The changes present in this biopsy are consistent with central core disease, which has been described in Great Danes in UK. Central core myopathy is considered to be an inherited condition. Clinical signs, which begin at about six months of age, are reported to include progressive muscle wasting, exercise intolerance, general body tremors and collapse. I am not aware that the disease has hitherto been seen in Australia.
To our knowledge, IMGDhas only been reported in Fawns and Brindles.
Symptoms
Reported symptoms for IMGD include progressive muscle wasting, exercise intolerance, general body tremors and collapse.
In Taj's case, he has splayed front paws (outwardly facing), severe exercise intolerance which is probably connected to his severe heat stress, wobbly hind leg movements, inability to jump and at any speed over a walk he bunny hops i.e. his hind legs move together.
See videos.
Treatment(s)
There are no (medical) treatments. See the supplements and feeding regime for Taj that allowed him to live beyond dogs studied.
Also see info about humans:
http://home.goulburn.net.au/~shack/medications.htm
Progression of the disease
Dr Jim Anderson (Faculty of Veterinary Medicine, University of Glasgow) has a special interest in IMGD and responded to us about a study he was doing on IMGD in Great Danes:
"My impression is that dogs that present severely affected are euthanased after weeks/couple of months whilst those that present with a a relatively mild condition continue as they are. Some of the patients are still about having a reasonable quality of life many months or a number of years after diagnosis. Of the dogs we have follow up on about 25% are still with us. My guess this data is skewed towards the severely affected animals as they are most likely to have been through a diagnostic procedure. I suspect that there is quite a range in the severity of the clinical signs.
We do not have any really long term follow up but that probably reflects the nature of the study."
Read the precis and abstract of Dr Anderson's paper (May 2006) on this disease
Clinical definition of Central Core Myopathy
In humans (and other mammals) a variety of progressive, degenerative ‘muscle wasting’ disorders are collectively termed muscular dystrophy. The basis of each form of muscular dystrophy can be different – some involve neurological defects, others result from structural deficiencies in protein components of muscle tissue, and some are the consequence of metabolic disorders. In all cases of muscular dystrophy, some contribution (either great or small) is genetic and hence these disorders are often familial. In addition, a very few cases can be the result of spontaneous or de novo mutations in the genes that underlie the manifestations of the diseases.
Environmental factors (diet, exercise, exposure to certain chemicals) can also be important in influencing how genetic variations or mutations can result in disease severity.
People and dogs (and other mammals) have in common many diseases which have a genetic basis – our DNA and genes are very similar! Over the past two decades, the genetic defects responsible for a variety of human disorders have been first identified in animals exhibiting similar clinical features. In Taj’s case it is the other way around - Central Core Disease (CCD) in humans has been well studied and the gene responsible (RYR1 ) has been identified but CCM or IMDG in dogs has only recently been recognised.
In humans, mutations in a gene called RYR1 can result in at least three clinically-defined syndromes:
1. Malignant Hyperthermia (MH), seen as heat/exercise or anaesthetic intolerance
2. A form of severe skeletal malformation (properly called Spondylocostal Dystosis)
3. Central Core Disease (CCD) a form of muscular dystrophy.
The RYR1 gene produces a large protein involved in regulation of the flow of calcium in and out of muscle cells.
Diagnosis process
In Taj's case, diagnosis was confirmed after X-rays ruled out any skeletal problem such as hip dysplasia. He then went on to have a nerve and muscle biopsy which were sent off to the veterinary pathologist for analysis.
Under general anaesthetic, a small piece of muscle (and nerve) was cut out of his leg and sent to the veterinary pathologist. The pathologist stained the muscle tissue sample and then looked at the muscle cells under a microscope. They look for a dark stain which shows a core in the muscle cells. Hence the name of the disease – Central Core Myopathy.
It is possible that there has been misdiagnosis of other Great Danes - 'Wobblers' seems to be a disease that a number of Great Danes are diagnosed within Australia. While some symptoms may display in some similar ways, this is a completely separate disease.
Read the Case Study about Taj our vets have had published:
"Inherited myopathy in a Great Dane". SE Davies, DR Davies, RB Richards and WJ Bruce. Australian Veterinary Journal 2008;86:43–45.
Free to download from link: doi: 10.1111/j.1751-0813.2007.00202.x
Taj's diagnosis from the Veterinary Pathologist
Gross Pathology -Two splinted biopsies, one of muscle and the other of nerve.
Histopathology - The muscle biopsy contains several areas where there are numerous enlarged fibres with central clearing of myofilaments. The clear zones often have central nuclei and can be seen, in LS, to extend for short distances along the fibre. There are infrequent fibres with peripheral loss of myofilaments. Sub-sarcolemmal nuclei are enlarged adjacent to areas of myofilment loss, but elsewhere are normal. While most of the affected fibres appear to have enlarged diameters, there are occasional atrophic fibres in relatively unaffected bundles. There is an equivocal increase in perimysial connective tissue, but no additional cells are present in the muscle sections. The sections of peripheral nerve show normal morphology.
Diagnosis - Focal myofibrillar lysis (muscular dystrophy)
Comments - The changes present in this biopsy are consistent with central core disease, which has been described in Great Danes in UK. Central core myopathy is considered to be an inherited condition. Clinical signs, which begin at about six months of age, are reported to include progressive muscle wasting, exercise intolerance, general body tremors and collapse. I am not aware that the disease has hitherto been seen in Australia.