Fibrinoid Leukodystrophy (Fibrinoid Encephalomyelopathy/Alexander Disease/Rosenthal Fiber Myelopathy)
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 may worsen over time
Clinical signs may worsen over time
Clinical Signs:
Behavioral/Mental Awareness
Alterations in personality or behavior
Aversion to touch
Reluctance to move
Vocalization abnormalities
Posture and Appearance
Stiffness
Tremor
Head tilt
Opisthotonos
Reddened mucous membranes
Movement
Ataxia
Tetraparesis
Drifting to the side
Myotonic spasms
Proprioception
Proprioceptive deficits
Cranial Nerves
Reduced menace response
Decreased physiologic nystagmus
Difficulty swallowing
Spinal Reflexes
Reduced to absent reflexes
Special Functions (e.g. respiration; urination)
Urinary incontinence
Respiratory failure
Other
Increased body temperature
Behavioral/Mental Awareness
Alterations in personality or behavior
Aversion to touch
Reluctance to move
Vocalization abnormalities
Posture and Appearance
Stiffness
Tremor
Head tilt
Opisthotonos
Reddened mucous membranes
Movement
Ataxia
Tetraparesis
Drifting to the side
Myotonic spasms
Proprioception
Proprioceptive deficits
Cranial Nerves
Reduced menace response
Decreased physiologic nystagmus
Difficulty swallowing
Spinal Reflexes
Reduced to absent reflexes
Special Functions (e.g. respiration; urination)
Urinary incontinence
Respiratory failure
Other
Increased body temperature
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
https://www.wisdompanel.com/en-us/dog-health-conditions/alexander-disease#:~:text=This%20disease%20is%20autosomal%20recessive,different%20genetic%20or%20clinical%20cause.
For
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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