Fibrinoid Leukodystrophy (Fibrinoid Encephalomyelopathy/Alexander Disease/Rosenthal Fiber Myelopathy)
Fibrinoid leukodystrophy is an extremely rare primary degeneration of nervous elements with a range of clinical signs and disease patterns.
Age of Onset: 3-6 months of age
Sex Predisposition: Any sex of animal can be affected
Clinical Course:
Clinical signs progress over the course of several weeks
Clinical signs progress over the course of several weeks
Clinical Signs:
Behavioral/Mental Awareness
Alterations in personality or behavior
Aversion to touch
Reluctance to move
Excessive fear
Depressed
Comatose
Vocalization abnormalities
Posture and Appearance
Head tilt
Opisthotonos
Movement
Paresis to plegia
Ataxia
Myotonic spasms
Tremor
Proprioception
Proprioceptive deficits
Cranial Nerves
Reduced menace response
Reduced to absent vestibulo-ocular reflex (physiologic nystagmus)
Spinal Reflexes
Can be normal or increased/decreased depending on the location of the lesion and the degree of spinal cord involvement
Special Functions (e.g. respiration; urination)
Urinary incontinence
Respiratory failure
Difficulty swallowing
Other
Increased body temperature
Reddened mucus membranes
Behavioral/Mental Awareness
Alterations in personality or behavior
Aversion to touch
Reluctance to move
Excessive fear
Depressed
Comatose
Vocalization abnormalities
Posture and Appearance
Head tilt
Opisthotonos
Movement
Paresis to plegia
Ataxia
Myotonic spasms
Tremor
Proprioception
Proprioceptive deficits
Cranial Nerves
Reduced menace response
Reduced to absent vestibulo-ocular reflex (physiologic nystagmus)
Spinal Reflexes
Can be normal or increased/decreased depending on the location of the lesion and the degree of spinal cord involvement
Special Functions (e.g. respiration; urination)
Urinary incontinence
Respiratory failure
Difficulty swallowing
Other
Increased body temperature
Reddened mucus membranes
The classic histologic lesion consists of so-called Rosenthal fibers distributed around the vessels in the white matter, subpial, and subependymal areas.
Suspected autosomal dominant mutation in canine orthologue of glial fibrillary acidic protein (GFAP) gene
For breed specific genetic testing, follow the link below:
https://www.wisdompanel.com/en-us/dog-health-conditions/alexander-disease#:~:text=This%20disease%20is%20autosomal%20recessive,different%20genetic%20or%20clinical%20cause.
For breed specific genetic testing, follow the link below:
https://www.wisdompanel.com/en-us/dog-health-conditions/alexander-disease#:~:text=This%20disease%20is%20autosomal%20recessive,different%20genetic%20or%20clinical%20cause.
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