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
Depressed
Comatose
Vocalization abnormalities
Posture and Appearance
Tremor
Head tilt
Seizure-like activity
Thoracic limb extension
Opisthotonos
Reddened mucus membranes
Stiffness
Difficulty swallowing
Movement
Paraparesis
Ataxia
Generalized weakness
Incoordination
Drifting to the side
Myotonic spasms
Flaccid tetraparesis
Proprioception
Knuckling
Falling
Knuckling on all limbs
Slow postural reflexes
Cranial Nerves
Reduced menace response
Decreased physiologic nystagmus
Spinal Reflexes
Increased spinal reflexes
Absent spinal reflexes in hindlimbs
Reduced spinal reflexes in forelimbs
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
Depressed
Comatose
Vocalization abnormalities
Posture and Appearance
Tremor
Head tilt
Seizure-like activity
Thoracic limb extension
Opisthotonos
Reddened mucus membranes
Stiffness
Difficulty swallowing
Movement
Paraparesis
Ataxia
Generalized weakness
Incoordination
Drifting to the side
Myotonic spasms
Flaccid tetraparesis
Proprioception
Knuckling
Falling
Knuckling on all limbs
Slow postural reflexes
Cranial Nerves
Reduced menace response
Decreased physiologic nystagmus
Spinal Reflexes
Increased spinal reflexes
Absent spinal reflexes in hindlimbs
Reduced spinal reflexes in forelimbs
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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