Hypomyelinating Leukodystrophy (Congenital Tremor Syndrome)
Hypomyelination syndrome is characterized by failure to generate a normal myelin sheath-axon relationship within the central nervous system. German Shepherd Dogs present with a unique congenital abnormality that involves primary hypomyelination of the white matter of the brain and spinal cord.
Other terms for this syndrome include “shaker pup”, “congenital tremor”, “shaker”, and “trembler” syndromes
Other terms for this syndrome include “shaker pup”, “congenital tremor”, “shaker”, and “trembler” syndromes
Age of Onset: Signs are present since birth or noticed in 2- to 3-week-old animals before weaning
Sex Predisposition: Any sex of animal can be affected
Clinical Course:
Clinical signs are non-progressive and affected animals may improve over time
Clinical signs are non-progressive and affected animals may improve over time
Clinical Signs:
Behavioral/Mental Awareness
Lip-smacking behavior
Tonic-clonic seizures/epilepsy
Posture and Appearance
Wide-based stance
Side-to-side truncal movements
Movement
Generalized muscle tremors
Hypermetric thoracic/pelvic limb ataxia
Dysmetria
Head bobbing
"Bunny-hopping"
Hopping gait
Dragging of limbs
Proprioception
Proprioceptive deficits
Spinal Reflexes
Reduced spinal reflexes
Cranial Nerves
Aberrant eye movement
Behavioral/Mental Awareness
Lip-smacking behavior
Tonic-clonic seizures/epilepsy
Posture and Appearance
Wide-based stance
Side-to-side truncal movements
Movement
Generalized muscle tremors
Hypermetric thoracic/pelvic limb ataxia
Dysmetria
Head bobbing
"Bunny-hopping"
Hopping gait
Dragging of limbs
Proprioception
Proprioceptive deficits
Spinal Reflexes
Reduced spinal reflexes
Cranial Nerves
Aberrant eye movement
White matter of the brain and spine
Suspected autosomal recessive inheritance
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