**Acknowledgements**

**5. Human T-lymphotropic virus type 1 (HTLV-1) associated** 

members including a case of SPG3A (**Figure 2**, IV-6).

HTLV-1 associated myelopathy (HAM) is a slowly progressive thoracic myelopathy characterized by spastic paraplegia with sensory and autonomic dysfunctions [17, 18]. There are many patients in the Kyushu district, the southwest part of Japan, because the prevalence rate of HTLV-1 carriers is high in Kyushu [18]. We found a HAM patient among the family

A 54-year-old man was admitted to our hospital for the further examination of gait disturbance. He first noticed the gait disturbance about 10 years ago. He walked without a heel and had urinary incontinence for 3 years. He pointed out increased deep tendon reflexes of his legs, a positive Babinski sign, and diminished deep sensation of the legs. On examination, increased leg spasticity was observed bilaterally (**Table 1**). Anti- HTLV-1 antibody was positive in both serum and cerebrospinal fluid (CSF). Serum from his healthy 91-year-old father (III-1, **Figure 2**) was also anti-HTLV-1 antibody positive, probably due to blood transfusion.

DNA were observed in his CSF. Whole-exome sequencing of his DNA did not identify pathogenic variants of the SPG3A and other SPG genes. We treated him with oral prednisolone [19],

Multiple sclerosis (MS) is a neuroinflammatory disorder involving the spinal cord, optic nerve and brain, and is prevalent in young women. It takes relapse and remission courses. The characteristic finding is multiple lesions in the brain and spinal cord observed on MRI [20]. Neuromyelitis optica (NMO) is a similar disease to MS, but usually long cord lesions (>3 vertebral body) are observed on spinal MRI, and autoantibodies against aquaporin 4 are usually

A 47-year-old woman was admitted to our hospital because of gait disturbance, clumsiness and numbness of the bilateral hands. Neurological examination revealed leg spasticity, increased deep tendon reflexes of all extremities, extensor plantar responses and sensory disturbances of the bilateral upper extremities and trunk. Spinal MRI showed a central cord lesion at C2-C3 with mild enhancement (**Figure 4**). Multiple ovoid periventricular lesions were observed on brain MRI. However, clinically spasticity was presented in the clinical examination in the lower legs only. The anti-aquaporin 4 antibody was not detected in her serum. We started high-dose methylprednisolone pulse therapy and subsequently administered oral fingolimod for relapse prevention. Her symptoms gradually improved except for the leg spasticity. Then we tried to treat her with botulinum toxin injection to her legs and gait rehabilitation [2]. The

), elevated neopterin (49 pmol/ml), and positive HTLV-1 proviral

**myelopathy**

62 Essentials of Spinal Cord Injury Medicine

Mild pleocytosis (8/mm<sup>3</sup>

the symptoms did not worsen after that.

**6. Multiple sclerosis (MS)**

detected in patients' sera [21, 22].

modified Ashworth score for her legs improved from 3 to 2.

We greatly appreciate the *ABCD1* gene analysis by Professor Nobuyuki Shimozawa (Division of Genomics Research, Life Science Research Center, Gifu University).
