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
Excessive fear
Altered mentation
Vocalization abnormalities
Posture and Appearance
Head tilt
Seizure-like activity
Opisthotonos
Stiffness
Movement
Paraparesis to tetraparesis
Ataxia
Generalized weakness
Tremor
Drifting to the side
Myotonic spasms
Proprioception
Proprioceptive deficits
Cranial Nerves
Reduced menace response
Decreased physiologic nystagmus
Dysphagia
Spinal Reflexes
Hyporeflexia
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
Excessive fear
Altered mentation
Vocalization abnormalities
Posture and Appearance
Head tilt
Seizure-like activity
Opisthotonos
Stiffness
Movement
Paraparesis to tetraparesis
Ataxia
Generalized weakness
Tremor
Drifting to the side
Myotonic spasms
Proprioception
Proprioceptive deficits
Cranial Nerves
Reduced menace response
Decreased physiologic nystagmus
Dysphagia
Spinal Reflexes
Hyporeflexia
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.
Unknown
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