Phenotypic and genotypic heterogeneity in hereditary motor neuronopathy
type V: A clinical, electrophysiological and genetic study.
There are multiple potential types of peripheral nerve dysfunction associated with GBS (acute inflammatory demyelinating polyneuropathy, AIDP; acute motor and sensory axonal neuropathy, AMSAN; acute motor neuropathy, AMAN; acute sensory neuronopathy
, acute pandysautonomia and overlap syndrome) and criteria for electrodiagnosis vary with the subtype of GBS.
Role of the blink reflex in the evaluation of sensory neuronopathy
1992) Ciliary neurotrophic factor prevents degeneration of motor neurons in mouse mutant progressive motor neuronopathy
The post-irradiation lower motor neuron syndrome neuronopathy
associated with Adenocarcinoma of Esophagus.
The author was initially given two synonyms for SBMA by a physician: Kennedy disease and X-linked bulbospinal neuronopathy
After a lifetime of suffering a variety of health problems, two-year-old Joshua Morgan has been diagnosed with motor neuronopathy
5) described a case of JCV granule cell neuronopathy
in a patient with JCV-associated cerebellar degeneration with isolated cerebellar symptoms.
Histopathologic examination of sections from the cortex, hippocampus, thalamus, hypothalamus, midbrain, pons, medulla, and cerebellum showed polioencephalitis affecting predominantly the diencephalon and brainstem and involving varying degrees of neuronopathy
, neuronal loss, astrocytosis, parenchymal and perivascular lymphocytic infiltration with CD45 immunopositivity, sparse macrophage activation, and axonal spheroid formation.
Approach to peripheral neuropathy and neuronopathy
4,5) Specific genetic tests are available for X-linked bulbospinal neuronopathy
(Kennedy's disease), which causes a slowly progressive lower motor neurone syndrome, sensory neuropathy, and partial androgen insensitivity leading to gynaecomastia and the recessive form of proximal spinal muscular atrophy which can occasionally come on in adult life.