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
Chewing
Salivation
Posture and Appearance
Wide-based stance of thoracic limbs
Side-side truncal movements in sternal recumbency
Oscillation of eyes
Aberrant eye movement
Chewing
Movement
Generalized muscle tremors
Hypermetric thoracic/pelvic limb ataxia
Dysmetria
Head bobbing
"Bunny-hopping"
Hopping gait
Dragging of limbs
Spinal Reflexes
Absent/delayed postural reactions
Decreased spinal reflexes
Other
Tonic-clonic seizures/epilepsy
Behavioral/Mental Awareness
Lip-smacking
Chewing
Salivation
Posture and Appearance
Wide-based stance of thoracic limbs
Side-side truncal movements in sternal recumbency
Oscillation of eyes
Aberrant eye movement
Chewing
Movement
Generalized muscle tremors
Hypermetric thoracic/pelvic limb ataxia
Dysmetria
Head bobbing
"Bunny-hopping"
Hopping gait
Dragging of limbs
Spinal Reflexes
Absent/delayed postural reactions
Decreased spinal reflexes
Other
Tonic-clonic seizures/epilepsy
White matter of the brain and spine
Suspected autosomal recessive inheritance
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