Granulometous Meningioencephalomyelitis (GME)


Granulometous meningioencephalomyelitis (GME)

Neurological Problems

Granulometous meningioencephalomyelitis

Comments: I am looking for information on Granulomatous meningo encephalitis. Our shitz tsu Ollie is suffering from the above. He has had it now for almost a week, it came on very suddenly after being very active and fit, it came on within hours. He is now at a vetenary surgery paralysed and showing all the signs of meningitis, the vet is excellent and we have no complaints, but wish we could understand this illness more and what are the success rate for a recovery and how long does it normally take before the dog is up on his feet again

Answer: John- Granulometous meningoencephalomyelitis (GME) comes in three forms: localized, generalized and ocular. Localized GME refers to the fact that a discrete area of the brain is affected. It can cause problems in a single activity or more generalized problems, depending on the exact area of the brain involved. Generalized GME means that there is wide involvement of the brain tissue (diffuse damage). There are usually signs of several neurologic problems occurring at one time with this form of the disease. Ocular GME affects the eyes, causes blindness and may or may not progress to one of the other forms. Localized and ocular GME have a better prognosis than generalized GME. Dogs with local or ocular GME may live for years. Localized GME is often responsive to corticosteroids for very long times, as well, so the dog may have a good quality of life in addition to a longer life. Ocular GME doesn't seem to respond well to treatment based on limited experience with it -- but most dogs adjust to visual impairment pretty well so this may not be an important issue. Unfortunately, generalized GME has a poor prognosis. The symptoms will sometimes resolve, or nearly resolve, pretty rapidly with therapy but it is necessary to continue using immunosuppressive medications, usually corticosteroids, life long. In most cases, the disease will become unresponsive to medications within a few weeks to several months and most pet owners opt for euthanasia when the signs become progressive and stop responding to therapy. When paralysis is present the most likely areas of the brain to be affected are the brain stem, but the cervical spinal cord can also sometimes be involved in the disease process. If the brain stem is involved, some dogs will be severely depressed or can even become comatose, if the response to therapy isn't good. The differential diagnosis list for GME is pretty short. It can be confused with other forms of encephalitis (infectious, toxic) and with cancers affecting the brain. Hepatic encephalopathy can look a lot like GME but usually there are obvious signs of liver disease which make it possible to distinguish that toxic effects of liver disease are causing the neurologic signs. There is no sure way to diagnose GME other than biopsy samples of the brain, which isn't a common procedure in veterinary medicine. Cerebrospinal fluid analysis can be helpful in ruling out other problems and hinting at GME. Computerized tomography (CT) scans or MRI scans can help rule out cancer and can help in identifying focal (local) areas of brain involvement. Both local and generalized GME usually have to be treated continuously and treatment can not generally be withdrawn without a recurrence of symptoms. Generalized GME is almost always fatal. In most cases the prognosis for generalized GME is poor, but I have gotten at least one letter complaining that I was too pessimistic in my opinion of GME, based on a case in which one person's dog had been living with the disease well controlled for over a year. It is usually necessary to use high doses of prednisone or other corticosteroids to get the symptoms to resolve but it is sometimes possible to use lower dosages for maintenance. An effort should be made to see if a lower dose will control the signs once they are well under control. Radiation therapy has been reported to help in a limited number of cases of GME. I hope that this information is useful to you. I understand that sometimes people subscribe when they really want to hear better news than this. We will refund the subscription price under these circumstances if a request to do so is made. Mike Richards, DVM 10/12/2003

Granulometous meningioencephalomyelitis (GME) in dogs

Question: Dear Dr. Mike, First let me say that you have a wonderful site. It has been extremely informative. I have a question I would like to ask you about our 3 year old lab named Abbey. We adopted Abbey when she was 9 months old, we have her brother Arlo since he was 10 weeks old. Abbey was diagnosed with mild hip dysplasia back in 99. We brought her in to a surgeon who thought that her hips were not bad and we should treat her with aspirin as needed. This is what we have been doing for the past 2 years without any problems. She is an avid swimmer, so we focus more on the swimming as her preferred method of exercise and less on the running. A little over a month ago Abbey began to have problems walking. She exhibited neurologic signs with her left rear leg. She would easily lose her balance and had difficulty righting her foot when turned over on the dorsal side. We brought her in to our veterinarian's office and too make a long story short she was treated with steroids (prednisone 10 mg 2x a day and received a shot) in addition blood was drawn to test for Lyme disease as well as a blood workup. She was also treated with deoxycycline and her hips were later x-rayed. X-rays determined that her hips had deteriorated immensely. Test results were negative on Lyme and the blood workup was normal. We schedule an appointment with a surgeon (the one we had seen previously) within a week and when he saw her the neurological signs had dissipated. His recommendation was to treat her with etogesics and get her off the steroids and re-evaluate in a month. We switched her med. and within a week she began to deteriorate, to the point of trembling when you would approach her to pet her. At this time she was still on the doxycylcline. We then switched her to Rimydil, she was like a new dog. However within a week she began to exhibit neurological symptoms (ataxia and conscious proprioception) again with both front and hind limbs especially on the left side. She also will turn her front left foot in to her body about 90 degrees or more when sitting or standing. Once again we brought her to our veterinarian who gave her steroids again and we also brought her back to the surgeon. She was diagnosed by both as being neurologic and the neurologist on staff was brought in to evaluate her. The neurologist examined her and her reflexes seemed normal (eyes ,gag reflex, etc.) In addition x-rays of her spine as well as a MRI were done. All came back negative. The MRI was done only of the spine and not the brain. In addition, spinal fluid was taken. The CSF results came in last week. She tested positive for distemper, high protein, low cell number the majority were lymphocytes (70-80%). She is now on dexamethasone 250ug 3 x a day, and as of this past saturday she is on the 2x a day. We also had blood drawn and we are testing her for a number of tick borne diseases (erlichiosis, RMSF, etc.) as well as toxoplasma and neospora. The neurologist thinks that this is either distemper or it could be GME. She seems to be responding to the steroids. She is still neurological but not as bad as she was. Her spirits seem better, we decided to let her go swimming since she seemed so down in the dumps. I think it is important for her to have a good quality of life even if it is short. I am interested in your opinion of what this might be. Also what other things we could test for. From my understanding of GME dogs may do well in the beginning when initially given steroids but they will fail given enough time. She has not had any seizures that we are aware of. Just the symptoms I have described, ataxia and conscious proprioception. Her muscles in the rear legs have atrophied and she has a tendency to sway back and forth when standing still. We are willing to do anything to help her. Do you think this could be distemper? She has had all of her vaccinations and we have her medical records from her previous owners who also kept her shots up to date. Both she and her brother are always vaccinated together, yet he has had no problems. If this is GME what are her chances of survival? Thank you for taking the time and reading this? We really appreciate any insight you may have.


Answer: Deb-

Granulometous meningoencephalomyelitis (GME) is an disorder of unknown origin that occurs in dogs. At the present time I think that the most likely cause is an immune mediated disorder but that doesn't narrow down the cause very much. It can occur in any age dog and is more common in female dogs and dogs of poodle or Airedale stock. In our practice we have seen this disease most commonly in chow chows but they aren't reported to be especially predisposed in the literature.

This disorder can cause a lot of different neurologic signs depending on how wide spread the brain or spinal cord involvement is but we have most commonly seen unexplained pain, sensitivity to touch, reluctance or inability to move or at least to move normally, and loss of proprioceptive reflexes such as the foot placing reflexes that you have noticed are not correct in Abbey's case. Signs can come on really suddenly or fairly rapidly over the course of a few weeks.

I don't know of a good way to diagnose this problem but on central spinous fluid (CSF) taps, the fluid usually has increased numbers of white blood cells with predominantly mononuclear cells, increased protein and sometimes increased pressure. Unfortunately this pattern is also seen with several other conditions, including distemper, Rocky Mountain spotted fever, bacterial meningitis and steroid responsive meningitis, so it isn't diagnostic. In people, vasculitis is reported to cause signs similar to GME but I don't know if this problem occurs in dogs or if it does how common it is. Some brain tumors can also cause inflammatory changes in the CSF.

The only treatment that I know of for GME is prednisone or other corticosteroid use. Dogs do respond pretty well to corticosteroids when GME is present, but they also would help with vasculitis, steroid responsive meningitis, distemper (sometimes), RMSF (sometimes) and probably the brain tumors, at least for a while.

In GME corticosteroids may induce remission of clinical signs that lasts for a few days or lasts for years. I can't recall seeing a study that gave a median or average survival time for this condition but my impression is that you are basically correct in your assessment of the how well steroids work. In the study I could find on this, Karen Munanan et. al, June 15 1998 Journal of the AVMA, the reported minimum survival time was one day and the longest just about 4 years. In that study dogs that had focal GME (affecting limited regions of the body) did better than dogs with generalized GME and radiation therapy also seemed to extend lifespan, although that works best for localized GME too, as far as I know. So corticosteroids work for some time but there is no way to tell how long it might be in advance.

Hopefully, if GME is the problem, Abbey will respond well and continue to respond well. I don't think that you can rule out distemper as a possible cause of the symptoms because you don't know her early history well, having adopted her at 9 months of age. The other disorders are possible but seem less likely than these two, especially with the neurologist's opinion favoring them, as well.

It is probably best to keep working with the neurologist as it is often possible to make a more precise diagnosis with time, as the symptoms change and the response to medications can be assessed. Good luck with this.

Mike Richards, DVM 6/27/2001

Granulometous meningioencephalomyelitis

Question: Dr. Richards,

My pet Lucy, a Lhasa Apso recently died of what is called granulometous meningoencephalitis. She was only six months old. Her illness seemed to come on so suddenly; within a day she went from being seemingly healthy and energetic to being extremely sick. The veterinary hospital, said there was no sign of virus, infection or bacteria they said it was an auto-immune disease.

I would first like to know any info on this disease/illness - What if any known causes can there be for this? What percentage of Lhasas die from this, especially at such a young age? Can this be the result of bad breeding? If so, how so?

Please let me know any info you can give me. Andrew

Answer: Andrew -

Granulometous encephalomyelitis is a disease that has only been reported in dogs, to the best of my knowledge. Poodles and terriers are supposed to be the most commonly affected breeds, but I am not aware of any information confirming a genetic predisposition to this disease. It most commonly occurs in middle aged dogs, usually small breeds. There is no known cause at this time but there are some theories, including an immune system defect, an unidentified infectious agent or an aberrant form of cancer. I think that the majority opinion is that this is an immune-mediated disorder.

There are three recognized forms of this disease, focal (signs all appear to be due to a disorder affecting only one area of the brain), diffuse (many different neurologic signs) and ocular, with no central nervous system component.

Granulometous encephalomyelitis usually comes on quickly. In the diffuse form, which I think is the most common form, there are behavioral changes, seizures, vision disturbances, sometimes balance problems or difficulty walking. The localized and ocular (eye) forms have specific signs associated with the affected area.

There is no cure that I know of. Corticosteroids, such as prednisone, are used to delay the progression of the disease but are not highly effective for this, with most dogs succumbing to the disease within a few months.

I have not found any information specific to lhasa apsos at this time. I will keep your need for specific information in mind and try to post anything I find.

I did not find any information pertaining to early onset of signs as a differentiating factor in possible causes, either. If you run across any information and need clarifications on it, please feel free to ask further questions.

Mike Richards, DVM 6/4/2000

GME Follow Up

Q: Dr. Mike: Thanks so much for your prompt response. It was very informative to the extent of my understanding. I have been in contact with the vets and specialist that treated Samain (Schipperke) and it is not recommended that she be given a MRI. He feels she may not come out of it this time. He strongly suggests that taking this medication, we should give it a week to 10 days to see how she responds. Then we should be able to tell more.

After hearing a number of comments on this GME, it seems not much is known as to the cause of it. Is there a chance it could be something else? And if not a tumor but GME, do you feel there is a cure for it?

A: I didn't want to get into prognoses in the last email, because I don't have a lot of personal experience and the literature paints a pretty bleak picture. Most cases of granulometous meningioencephalitis (GME) are fatal within a a few months to a year. The diseases that look most like GME are brain tumors and reticulosis. The prognosis for them is similar. There are some other problems that could be present and may have better prognoses. Lead poisoning can produce signs close to GME. Other heavy metal toxins can do this, too. Severe liver disease and kidney disease can produce neurologic signs. I am pretty sure that your vet and the specialist thought of most of these things, too. The toxins can be difficult to detect without specific blood testing but there are usually either reasons to consider them or other signs present. Some vets have associated hypothyroidism with severe central nervous system signs but I have not personally seen this occur.

I hope that your dog will be the exception to the poor prognosis.

Mike Richards, DVM


Michael Richards, D.V.M. co-owns a small animal general veterinary practice in rural tidewater Virginia. Dr. Richards graduated from Iowa State University's College of Veterinary Medicine in 1979, and has been in private practice ever since. Dr. Richards has been the director of the PetCare Forum...