•Necrotizing Leukoencephalitis (NLE)
•Synonyms: necrotizing encephalitis of the Yorkshire terrier
•Breeds: Yorkshire Terrier , Chihuahua , Shih-Tzu, slightly older dogs (1 to 10 years old)
•Clinical features: Multifocal localization (forebrain and brainstem), central vestibular disease common, chronic progressive
There are no tests to definitively diagnose these diseases other than direct brain biopsy or autopsy examination. Many reports on GME and necrotizing encephalitis suggest that these are inevitably progressive and fatal disorders. These studies are usually based on autopsy reports and therefore all examined animals have died from the disease. We have seen a large number of animals with idiopathic encephalitis respond to immunosuppression and so a final diagnosis cannot be made. GME and necrotizing encephalitis may simply reflect one of the most severe forms of immune-mediated encephalitis. There has also been some speculation that GME may represent a form of the cancer lymphoma.
Granulomatous Meningoencephalitis
This common inflammatory brain disease in dogs is abbreviated GME. The cause is unknown. Female dogs of small breeds, especially terriers, Dachshunds, Poodles, and Poodle crosses, are predisposed. Although GME can occur at any age, most affected dogs are 2 to 6 years of age.
A chronic form of GME called Pug encephalitis occurs as an inherited disease in Pugs between the ages of 9 months and 4 years. It often begins with seizures, confusion, and loss of memory. This form of the disease has also been seen in Yorkshire Terriers and Maltese.
GME can affect all parts of the brain (the disseminated form), or only specific areas (the focal form). There is a rare ocular form that targets the optic nerves of the eyes.
The disseminated disease appears suddenly and progresses over a matter of weeks. It is characterized by incoordination, stumbling, falling, circling, head tilt, seizures and, dementia.
The focal disease begins with symptoms such as those of a brain tumor. Behavior and personality changes may predominate. The focal disease progresses to the disseminated disease over a period of 3 to 6 months.
The ocular disease is characterized by sudden blindness with a dilated pupil. It progresses to the disseminated disease more slowly than does the focal disease.
GME can be suspected when a toy dog such as a Poodle inexplicably develops confusion, disorientation, seizures, or other neurological signs that progress rapidly over a matter of weeks. A spinal tap with analysis of cerebrospinal fluid helps confirm the diagnosis. A CAT scan or MRI is useful in determining the form and location of the disease.
Treatment: Corticosteroids and immunosuppressive drugs may slow the progression of GME and provide temporary relief for several months. However, GME is almost invariably a progressive and fatal disease.
Necrotizing encephalitis (NE) is a category of noninfectious inflammatory encephalitides that includes two pathologically distinct disorders – necrotizing meningoencephalitis (NME) and necrotizing leukoencephalitis (NLE). These are thought to represent autoimmune disorders. Both disorders are similar in that they are characterized by multiple cavitary necrotic nonsuppurative inflammatory brain lesions that involve both gray and white matter. It is very possible that NME and NLE represent variants of the same disease process.
In NME, the lesions are typically found in the cerebrum, with consistent involvement of the leptomeninges. Extensive cerebral cavitations with a loss of demarcation between gray and white matter are typical for NME. Although Pug and Maltese dogs are most commonly affected by NME, other breeds reported with this disorder included Chihuahua, Shih Tzu, Pekingese, Papillon, and Boston Terrier.
Necrotizing leukoencephalitis (NLE) is characterized by similar lesions that often involve the brain stem in addition to the cerebrum, with less consistent involvement of the leptomeninges and cerebral cortex (i.e., mainly white matter). Yorkshire Terriers appear to be the most common breed afflicted by NLE, but this disease may also affect other small-breed dogs (reports include Pugs, Maltese, Chihuahuas, French Bulldogs).