What is the beagle pain syndrome

Artery Inflammation in Dogs

Juvenile Polyarteritis and Beagle Pain Syndrome in Dogs

Juvenile polyarteritis, also referred to medically as beagle pain syndrome, is a systemic disease that is seemingly of genetic origin, affecting only certain breeds. It is most commonly reported in young beagles, although a similar syndrome has been reported in other breeds, most notably boxers and Bernese mountain dogs. This disease is rare, and can be defined as a simultaneous inflammation of an artery, or several arteries, with irritation, or infection, of the small vessels in the spinal cord in the neck and in the heart.

Symptoms of the condition seem to come and go, with indications that suggest a serious bacterial infection: high fever, pain, and a high white blood cell count. This common misdiagnoses makes juvenile polyarteritis difficult to treat, since antibiotics have no effect. If your veterinarian does not suspect beagle pain syndrome, and your pet is showing indications of this condition, it would be wise to ask your doctor to consider it. Especially if your dog has already gone through a course of antibiotics. This condition can also be referenced as necrotizing vasculitis: inflammation and tissue death of a vessel.

Symptoms and Types

• Neck pain
• Stiff neck
• Lowered head
• Hunched back
• Grunting when lifted
• Muscle spasms (especially in front legs and neck)
• Shaking
• Fever
• Lack of appetite
• Lethargy
• Unwillingness to move

For a beagle puppy that is suffering from this condition, opening the jaw will seem to be painful, and the puppy will be reluctant to bark. The symptoms usually will become apparent when the puppy is four to ten months of age, but the condition can manifest at an older age as well. It may resolve itself without treatment, but even if it does, it will typically return within a few months.

Causes

A latent genetic factor is believed to be one of the causes for juvenile polyarteritis, since only some breeds are susceptible. Medical researchers also suspect a related auto-immune factor.

Diagnosis

Bacterial meningitis, inflammation of a vertebral disc, spinal tumor, and cervical disc disease should be ruled out before confirming a diagnosis of juvenile polyarteritis. X-rays tend not to show evidence of disease if necrotizing vasculitis is present. A spinal tap is usually better for determining the nature of the disease. While this is a form of meningitis, its source is not bacterial, so antibacterial medications will not resolve the condition.

Your veterinarian will need to conduct a full blood work-up for further confirmation, and the lab results may show anemia (low red blood cells), a high white blood cell count indicating infection, or other blood abnormalities. There will usually be an attendant fever, also an indication of infection.

Treatment

Prednisone, an anti-inflammatory, immunosuppressive medication similar to cortisone, is the usual treatment of choice. Patients tend to show rapid improvement in just a few days, but even so, relapses often occur when the medication is discontinued. Continuing treatment for a longer period of time, like six months, will sometimes result in permanent resolution. At the outset of therapy, steroid treatment should be administered at a level that will produce a remission of the symptoms, and then your veterinarian can recommend a regimen of oral therapy. Over the course of treatment, the amount will be reduced slowly to the lowest possible dose needed to control symptoms. If symptoms return, steroid treatment will need to begin again.

Living and Management

One of the side effects of steroid treatment is fluid retention and increased thirst. To prevent accidents or discomfort on your dog's behalf, you will need to take your dog out frequently for urination, even if only for a small amount. A calm, quiet environment, where your pet will not be stimulated, is important. Moving will be painful during the recovery process, and it will benefit your dog if you give it an isolated space, away from children or animals, at least until the symptoms have subsided. Even after recovery, you will need to be alert to the possibility that your dog may have a relapse.

Steroid-responsive meningitis-arteritis (SRMA)

Synonym(s): Necrotizing vasculitis, Beagle pain syndrome, aseptic suppurative meningitis, canine pain syndrome, canine juvenile polyarteritis syndrome.

Introduction

• Common(est) meningitis, usually <2 year old animals of larger breeds.
• Also known as necrotizing vasculitis, Beagle pain syndrome, aseptic suppurative meningitis, canine pain syndrome, canine juvenile polyarteritis syndrome.
• Signs: mimic cervical disk extrusion.
• Breed: specific syndromes in Bernese Mountain Dog, Beagle, Boxers, Weimeraners, and Nova Scotia duck tolling retreivers.
• Diagnosis: signs, CSF.
• Treatment: corticosteroids.
• Prognosis: may be acute or have relapsing pattern.

Presenting signs

• Animal may be reluctant to move.
• Lethargy, anorexia.
• Cervical rigidity and pain.
• Animal may move 'carefully' to avoid jarring.
• Pyrexia.

• Signs may be intermittent initially and progress to neurologic deficits, ie weakness, paralysis, blindness and seizures.

Acute presentation

• Lethargy, cervical rigidity, pain, stiff gait, pyrexia.
• Hyperesthesia along the vertebral column.
• Hunched posture with profound guarding of the head and neck.

Chronic presentation

• Less common.
• May be observed following relapses of acute disease and/or inadequate treatment.
• Paresis, ataxia.

Age predisposition

• Typically 6 and 18 months with a range from 4 months to 7 years.

Breed/Species predisposition

• Large breed dogs are primarily affected.
• BeagleBeagle.

Cost considerations

• Medication: prednisolone +/- anticonvulsants.

Special risks

• General anesthesia: monitor oxygen supply carefully because hypoxia due to seizure activity and airway compromise cytotoxic brain edema and possibly increased intracranial pressure.

Pathogenesis

Etiology

• Immunopathologic basis suspected.
• Idiopathic.
• Disease is believed to be heritable.
• Activated T cells have been demonstrated in dogs with SRMA indicating potential contact with an antigenic stimulus.
• A Th2-mediated immune response is most likely based on the presence of high CD4:CD8a ratios and a high proportion of B cells in peripheral blood and CSF.

Predisposing factors

General

• Frequent exposure to monovalent attenuated live virus vaccines may sensitize patient to viral antigen and increase the risk of developing an immune-mediated reaction.

Pathophysiology

• Cerebral vessel injury due to immunologic disease deposition of immune complexes in vessel walls activation of Hageman Factor activation of complement, kinin, and plasma systems chemotaxis of neutrophils enhanced vessel wall permeability fibrin deposition, thrombosis and necrosis.
• Clinical signs result from a combined meninigitis and arteritis of leptomeningeal vessels.
• The arteritis also may involve the vessels of the heart, mediastinum and thyroid glands.

Timecourse

• Slowly progressive course over several months
• Animals may experience cyclic bouts of the disease with each bout lasting 5-10 days with intervening periods of complete or partial normalcy lasting at least one week.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

• Recent references from PubMed and VetMedResource.
• Eminaga S, Cherubin G Bet al(2013)STIR muscle hyperintensity in the cervical muscles associated with inflammatory spinal cord disease of unknown origin.JSAP 54(3), 137-142 PubMed.
• Rose J H, Harcourt-Brown T R (2013)Screening diagnostics to identify triggers in 21 cass of steroid-responsive meningitis-arteritis.JSAP54(11), 575-578 PubMed.
• Tipold A, Schatzberg S J (2010)An update on steroid responsive meninigitis-arteritis.JSAP51(3), 150-154 PubMed.
• Lowrie M, Penderis Jet al(2009)The role of acute phase proteins in diagnosis and management of steroid-responsive meningitis arteritis in dog.Vet J182(1), 125-130 PubMed.
• Wrzosek M, Konar Met al(2009)Cerebral extension of steroid-responsive meningitis arteritis in a Boxer.JSAP50(1), 35-37 PubMed.
• Behr S, Cauzinille L (2006)Aseptic suppurative meningitis in juvenile Boxer dogs: retrospective study of 12 cases.JAAHA42(4), 277-282 PubMed.
• Burgener Iet al(1998)Chemotactic activity and IL-8 levels in the cerebrospinal fluid in canine steroid responsive meningitis-arteritis.J Neuroimmunol89(1-2), 182-190 KUNDOC.
• Haburjak J J, Schubert T A(1997)Flavobacterium breve meningitis in a dog.JAAHA33(6), 509-512 PubMed.
• Hess P R, Sellon R K(1997)Steroid-responsive, cervical, pyogranulomatous pachymeningitis in a dog.JAAHA33(5), 461-468 PubMed.
• Tipold A (1995)Diagnosis of inflammatory and infectious diseases of the central nervous system in dogs - a retrospective study.JVIM9(5), 304-314 PubMed.
• Tipold A, Vandevelde M,Zurbriggen A(1995)Neuroimmunological studies in steroid-responsive meningitis-arteritis in dogs.Res Vet Sci58(2), 103-108 PubMed.
• Maretzki C H, FisherD J,Greene C E (1994)Granulocytic ehrlichiosis and meningitis in a dog.JAVMA205(11), 1554-1556 PubMed.
• Tipold A, Jaggy A (1994)Steroid responsive meningitis-arteritis in dogs:long term study of 32 cases.JSAP35 (6), 311-316 VetMedResource.
• Poncelet L,Balligand M(1993)Steroid-responsive meningitis in three boxer dogs.Vet Rec132(14), 361-362 PubMed.
• Sorjonen D C (1992)Myelitis and meningitis.Vet Clin North Am Sm Anim Pract22(4), 951-964 PubMed.
• Meric S M (1988)Canine meningitis - a changing emphasis.JVIM2(1), 26-35 PubMed.
• Meric S M, Perman V, Hardy R M (1985)Corticosteroid responsive meningitis in ten dogs.JAAHA21(5), 677-684 VetMedResource.
• Easley J R (1979)Necrotizing vasculitis - an overview.JAAHA15(2), 207-211 VetMedResource.

Other sources of information

• Merck Veterinary Manual.(1998) 8th edn. Merck and Co Inc, p581.
• Taylor S M (1997)Bernese Mountain Dog steroid-responsive meningitis - polyarteritis.In:The 5 minute veterinary consult - canine and feline.p390.

Beagle Pain Syndrome: Our Vet Explains Steroid-Responsive Meningitis-Arteritis

The information is current and up-to-date in accordance with the latest veterinarian research.

Learn more

Steroid-responsive meningitis-arteritis (SRMA) was initially referred to as beagle pain syndrome. It was first identified in young laboratory Beagles that demonstrated clinical signs of lameness, pain, and fever. The condition has also been known by several other names, including juvenile polyarteritis syndrome, necrotizing vasculitis, panarteritis, and polyarteritis, amongst others.

The term SRMA is currently the most universally accepted name, as it refers to not only the underlying pathology (i.e., inflammation of the meninges and their associated arteries) but also the most widely used treatment and its success in managing this disease. The condition has also since been described in various other breeds of dogs, making the term beagle pain syndrome no longer appropriate. Learn more about SRMA and its signs and causes below.

What Is Steroid-Responsive Meningitis-Arteritis?

SRMA is an immune-mediated disease that some consider to be the most frequently diagnosed inflammatory disorder involving the central nervous system (CNS) in dogs. Two different forms of SRMA have been documented: acute and chronic.

As alluded to above, the name of this syndrome gives some valuable clues as to what pathology is involved. The disease is characterized by inflammation involving the meninges and associated arteries, along with evidence of this inflammation within the cerebrospinal fluid (CSF).

Most studies on SRMA have not identified a sex predilection; in other words, males and females appear to be at similar risk, although one study did report a higher prevalence in male dogs. Typically, the condition is identified in dogs under 2 years of age (95% of cases), with the peak prevalence between 6 and 18 months. There have, however, been reports of SRMA in dogs as young as 3 months and as old as 9 years.

What Are the Signs of Steroid-Responsive Meningitis-Arteritis?

Acute SRMA

The clinical signs seen can vary depending on the form of the disease present. Typically, the acute form is characterized by neck pain and rigidity or stiffness, which can be intermittent, along with a fever (and associated lethargy). Many dog owners describe the signs as having a waxing and waning coursethis is important to appreciate, given that when presented for examination at a veterinary clinic, dogs with SRMA may not be exhibiting all or even any of the signs commonly seen with this disease. For instance, while fever is common in dogs with SRMA, a normal temperature cannot rule it out as a potential diagnosis in a dog with concurrent neck pain, stiffness, and lethargy.

Chronic SRMA

The chronic form, which is considered less common, may also demonstrate signs seen with the acute form; however, it usually involves repeated episodes of neck pain accompanied by additional neurological deficits (e.g., weakness and an uncoordinated gait). These deficits are consistent with a spinal cord or multifocal neurological disorder and represent an extension of the inflammation from the meninges to adjacent structures (i.e., the spinal cord (myelitis) and the brain (encephalitis)).

Chronic lesions can include meningeal fibrosis (or scarring) and arterial stenosis (narrowing of arteries), which can obstruct normal CSF flow and even occlude vessels, respectively. Such lesions can lead to ischemia of the CNS parenchyma and the other neurological deficits described above. Thus, it can be difficult to distinguish the chronic form of SRMA from the more commonly identified meningoencephalitis of unknown etiology.

Other Signs and Diagnosis

Interestingly, various cardiac changes have also been identified in dogs with SRMA. In one population of 14 dogs, such changes were considered common. In humans, the co-occurrence of cardiac disease in patients with inflammatory CNS disease is well-described. While most cardiac changes identified in dogs with SRMA appear to resolve with steroid therapy, further research is required to determine if cardio-supportive treatment is necessary to avoid potential complications.

There is currently no definitive test for SRMA in a living dog. Thus, a diagnosis involves consideration of several variables, such as history and clinical signs, physical examination findings (e.g., neck pain and fever), the presence of nonspecific findings on laboratory work (blood and CSF), and excluding other potential diagnoses that can present similarly (e.g., infectious diseases, particularly in young dogs, and meningoencephalitis of unknown etiology or even neoplasia in older dogs).

What Are the Causes of Steroid-Responsive Meningitis-Arteritis?

The exact underlying cause is currently unknown. However, SRMA is understood to be an immune-mediated disease involving abnormal and dysregulated immune responses directed toward the central nervous system of specific breeds of dogs.

The reason or trigger/s behind such a response remains to be determined. No studies have identified an environmental, infectious, or neoplastic (cancerous) trigger for this disease. There is also no relationship between vaccination and the development of SRMA in dogs.

How Do I Care for a Dog With Steroid-Responsive Meningitis-Arteritis?

As the name suggests, treatment of this condition involves using steroids (otherwise known as corticosteroids or glucocorticoids) such as prednisone or prednisolone. Generally, dogs with SRMA are treated with prolonged courses of steroids, starting at immunosuppressive dosages and gradually tapering the dose (until the drug can be safely discontinued) over approximately 6 months. Such courses have proven excellent in achieving remission, with some studies reporting success in up to 98.4% of cases. Most dogs show clinical improvement within 2 days of starting steroid therapy.

Relapse

Unfortunately, in many dogs, this remission appears to be short-lived. Relapse rates range from anywhere between 16% and 47.5%. Relapses are believed to result from either inadequate dosage or an inappropriate or insufficient duration of treatment. Some authors have also proposed that certain dogs may be insensitive to steroids, as documented sporadically in humans undergoing treatment for various immune-mediated diseases. It has also been hypothesized that inadequate treatment leads to the development of the chronic form of SRMA.

Predicting which dogs will relapse and when is a problem that has prompted much research. Unfortunately, a predictive marker remains elusive, and relapses have been reported both during treatment and following cessation of therapy with steroids. Most cases that relapse experience one or two relapse episodes; however, although uncommon, some dogs have been noted to have three or even four relapses.

It may also be the case that certain breeds are more likely to suffer a relapse, with one study describing such a finding in Beagles and Bernese Mountain dogs. Older dogs appear less likely to relapse, with apparent resistance to recurrence of signs after approximately 2 years of age being described by some authors.

Not only has this high relapse rate prompted much investigation into a possible predictive marker, but it has also led to studies looking at the use of additional drugs in managing relapses to hopefully prevent further relapse. This is not surprising, given the multiple immunosuppressive drugs available in veterinary medicine and the somewhat common practice of using multimodal therapy to manage cases of inflammatory CNS disease in dogs.

One study looked at cytosine arabinoside, a chemotherapeutic, to help address such issues. While this addition did result in remission of signs in 10 out of 12 dogs, side effects and adverse events associated with its inclusion were identified in all 12 dogs, many requiring additional measures to manage these adverse events.

It is also worth mentioning that prolonged courses of steroids in dogs have also been associated with mild side effects, the most reported being diarrhea. These adverse effects are dose-related and therefore tend to be more apparent earlier in the treatment course, and large-breed dogs are also more susceptible.

Other Treatment Options

Another potential therapeutic option for dogs with SRMA is targeting the endocannabinoid system (e.g., using derivatives of Cannabis sativa). Endocannabinoids have proven helpful in immunomodulation, neuroprotection, and helping control inflammatory disorders of the CNS. A recent study showed upregulation of specific endocannabinoid receptors in dogs with SRMA, suggesting that targeting the endocannabinoid system may help manage dogs with SRMA.

What Is the Prognosis for a Dog With Steroid-Responsive Meningitis-Arteritis?

The prognosis varies depending on the form of SRMA a dog is diagnosed with. The acute form, especially in young dogs, generally has a good to even excellent prognosis with early implementation of steroid treatment.

In contrast, the chronic form usually has a more guarded prognosis and requires more aggressive and long-term therapy.

Frequently Asked Questions (FAQs)

What Breeds of Dogs Get SRMA? Does It Only Occur in Beagles?

While SRMA, formerly known as beagle pain syndrome, was first identified in Beagles, several other breeds have since been recognized as predisposed to this condition. Such breeds include Beagles, Bernese Mountain dogs, Border Collies, Boxers, Golden Retrievers, Jack Russell Terriers, Weimaraners, Whippets, and Wirehaired Pointing Griffons. Notably, no differences in disease severity, diagnostic findings, or even outcome have been recognized across predisposed breeds.

Is SRMA Contagious?

No. SRMA is an immune-mediated disease that stems from an abnormal immune response within the body. In the case of SRMA, this response is directed toward or against the meninges (the membranes that line the brain and spinal cord) and associated arteries. No underlying triggers have been identified that could lead to the abnormal immune response and clinical signs seen in dogs with SRMA.

Conclusion

In summary, SRMA is a common immune-mediated disorder identified in several dog breeds (not just the Beagle), particularly young dogs. Two forms of the disease have been well-described, and the clinical signs and prognosis differ. Treatment of dogs with SRMA is centered on using corticosteroids such as prednisone, which are highly effective in achieving remission of clinical signs, especially in dogs with the acute form of the disease. Unfortunately, relapse is very common and necessitates close monitoring in all dogs with a history of SRMA for the recurrence of signs and subsequent rapid re-implementation of steroid therapy.

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