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In order to make a correct diagnosis it is essential to distinguish between lower and upper motor neurone lesions.
Evoked motor potentials represent a new and reliable method to detect physiological abnormalities of central motor pathways early in the course of motor neuron disease.
That should be considered in the differential diagnosis of motor neuron diseases in order to start accurate therapy.
The lesions are those of a lower motor neuron disease which appeared to have a genetic cause.
Recognizing related motor neuron diseases will prevent misdiagnosis while allowing proper disease counseling.
The clinical observations are consistent with a primary abnormality of lower motor neuron function.
Depending on the associated clinical symptoms and signs, they may signify the presence of a variety of disorders of the lower motor neuron.
The present case and two reported similar cases may constitute a new subgroup of motor neuron disease.

Related Questions

Does ALS affect both upper motor neurons and lower motor neurons?5 answersYes, amyotrophic lateral sclerosis (ALS) affects both upper motor neurons (UMN) and lower motor neurons (LMN). ALS is a neurodegenerative disease characterized by the degeneration of UMN in the motor cortex and LMN in the brainstem and spinal cord. The degeneration of these two neuronal populations is somatotopically related, meaning that the affected regions are connected. In ALS, UMN degeneration is an early event that precedes LMN degeneration. The diagnosis of ALS requires recognition of both UMN and LMN dysfunction. Electromyography (EMG) is used to detect LMN involvement, while there are no generally accepted markers for monitoring UMN abnormalities. Therefore, a full clinical assessment is necessary to diagnose ALS and monitor disease progression.
How to perform a neurological examination?5 answersThe neurological examination is an essential part of the diagnostic process. It should be used as a form of hypothesis testing once a differential diagnosis has been formulated from the patient's history. The nervous system is more complicated than other systems, but a relatively simple system of examination can be used to test many important facets of its function. Not all patients need all tests of nervous system function, and patients with different neurological problems need different types of neurological examination. The examination includes testing high brain function, cranial nerves, sensory function, motor function, reflexes, coordination, gait, and autonomic function. The examination can be performed in two parts - selection of the important parts of nervous system function that need to be examined, and use of appropriate technical skills to perform each part of the examination competently. The examination can be adapted for different age groups, with observation and enticement of actions being used for infants and small children.
What is the evidence for schwann cell remyelination of cns lesions?4 answersSchwann cell remyelination of CNS lesions is supported by multiple studies. In spinal cord sections from patients with multiple sclerosis, P0 immunostaining, a marker for peripheral myelin, was observed in regenerating sheaths of peripheral nervous system origin. Transplanting human Schwann cells into demyelinated areas of rat spinal cords resulted in remyelination. Additionally, Schwann cell remyelination was found to be stable and not replaced by oligodendrocyte remyelination over time in ethidium bromide-induced demyelinating lesions in rats. It has also been suggested that Schwann cells protect central axons and prevent scar tissue formation, which could impede oligodendrocyte remyelination. Furthermore, transplantation studies indicate that Schwann cells that remyelinate CNS axons are likely generated from CNS precursors. These findings provide evidence for the involvement of Schwann cells in remyelination of CNS lesions.
How could you test for possible neuronal deficits?5 answers
Is Duchenne muscular dystrophy a lower motor neuron disease?12 answers
Is Muscular Dystrophy upper or lower motor neuron?6 answers

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