**1. Introduction**

Spinal cord disorders are induced by diseases of various categories: infections [1] (e.g. herpes zoster or human T-cell lymphotropic virus type 1), inflammation (e.g. multiple sclerosis [2]), vascular diseases (e.g. spinal cord infarction [3]), degeneration (e.g. amyotrophic lateral sclerosis [4]), genetic diseases (e.g. hereditary spastic paraplegias [5]), metabolic disorders [6], trauma, etc. These diseases involve spastic paraplegia or tetraplegia, abnormal sensation, bladder and anal dysfunction, etc. This chapter describes the medical etiologies and treatments for spastic paraplegias. Diagnostic and therapeutic aspects of spastic paraplegias due to non-traumatic

Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

© 2016 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons

spinal cord disorders will be described. In this chapter, cases with X-linked adrenoleukodystrophy (X-ALD), amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegia (HSP), HTLV-1-associated myelopathy (HAM), multiple sclerosis (MS) are introduced.

examination revealed saccadic eye movement, dysarthria, ataxia and spasticity of the bilateral feet, exaggerated deep tendon reflexes (DTRs), and bilateral positive Babinski signs. Blood examination disclosed elevation of very long chain fatty acids (C24:0/C22:0 2.09, C25:0/C22:0 0.080, and C26:0/C22:0 0.075) and ACTH (173 pg/ml). Brain MRI showed FLAIR hyperintensities in the cerebellar white matter and callosal body, whereas there was no gadolinium enhanced area (**Figure 1**). No atrophy or abnormal signals were observed on MRI of the spinal cord. Brain single photon emission computed tomography (SPECT) demonstrated cerebellar hypoperfusion. For an accurate diagnosis, gene analysis was performed by another institu-

Spastic Paraplegias Due to Non-Traumatic Spinal Cord Disorders

http://dx.doi.org/10.5772/intechopen.71457

59

He was administered hydrocortisone and propiverine because of his adrenal insufficiency and frequent urination, and underwent physical rehabilitation (walking and balance exercise) for his leg spasticity and ataxia. We referred him to another hospital, and allogeneic hematopoietic stem cell transplantation was recommended [9, 10], but he denied this treatment.

ALS is a fatal disorder characterized by muscle weakness and atrophy, and swallowing and respiratory disturbances [11]. The pathologic findings are upper (brain) and lower (spinal cord) motor neuron degenerations. In some ALS cases, spastic paraplegia can be a predominant symptom in the early stage of the disease. Here we present a case that showed spastic

A 60-year-old man was admitted to our hospital to alleviate his lower leg spasticity. Three years ago, he suffered from left leg discomfort and gait disturbance. Then the same sense of discomfort spread to his right foot. Neurological examination on admission showed marked leg spasticity with laterality and a spastic gait, exaggerated DTRs, and positive pathological reflexes. The brain and spinal cord MRI findings were normal. Motor evoked potentials suggested upper

We administered some muscle relaxants. He underwent gait rehabilitation and botulinum toxin injection to his lower legs. These therapies slightly improved the range of motion of knee and

After 1 year, he noticed dysphagia and intrinsic hand muscle atrophy. Neurological reevaluation revealed bulbar signs and distal muscle weakness, these findings leading to a diagnosis of ALS. Although he underwent intermittent edaravone infusion therapy [12], his

HSP is a genetic neurodegenerative disorder that involves bilateral leg spasticity with additional features: mental impairment, peripheral neuropathy, cerebellar ataxia, retinal

muscle weakness and atrophy gradually worsened and he became bedridden.

tion, which revealed a non-synonymous missense variant of the *ABCD1* gene.

**3. Amyotrophic lateral sclerosis (ALS)**

foot joints. But he refused intrathecal baclofen.

**4. Hereditary spastic paraplegia (HSP)**

paraplegia as an initial phenotype.

motor neuron disturbances.
