Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 67-year-old woman began to have difficulty in walking due to spastic paraparesis, sensory impairment in the lower limbs and instability on standing at the age of 59 years. Eight years later, she developed complete spastic paraplegia and urinary incontinence. On examination, she was found to have down-beat nystagmus and ocular hypermetria. Brain CT scan and MRI revealed cerebellar atrophy, predominantly in the vermis. HTLV-I antibody titer was increased in both serum and cerebrospinal fluid (CSF). She was diagnosed as having HAM and placed on prednisolone, salazosulfapyridine and high-dose vitamin C. Her symptoms including gait disturbance, nystagmus and ocular hypermetria improved but did not completely disappear. In parallel with clinical improvement, the antibody titer to HTLV and the ratio of CD4/CD8 lymphocyte subsets in blood decreased. The above observations suggest that the cerebellar signs and cerebellar atrophy share the same etiology with HAM. The presence of nystagmus and prominent cerebellar signs plus the CT and MRI findings strongly suggest that the lesions in HAM are not limited to the spinal cord but extend to the brainstem and cerebellum.
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PMID:[HTLV-I associated myelopathy (HAM) with cerebellar atrophy presenting a down-beat nystagmus]. 766 28

Cerebellar symptoms at onset are unusual in HTLV-I/II-associated tropical spastic paraparesis (TSP). A prospective study of neurological disorders in Panama (1985-1990) revealed 13 patients with TSP and 3 with HTLV-I/II-associated spinocerebellar syndrome (HSCS) presenting at onset loss of balance, wide-based stance and gait, truncal instability, and mild leg ataxia (vermian cerebellar syndrome), with absent upper limb dysmetria but with postural tremor, downbeat nystagmus, and dysarthria. In 4-5 years, spinal cord manifestations of TSP developed, including spastic paraparesis, pyramidal signs, bladder and sphincter disturbances. Two patients were infected with HTLV-I and another one, a Guaymi Amerindian woman, with HTLV-II. Magnetic resonance imaging (MRI) demonstrated cerebellar atrophy involving predominantly the superior vermis. Mild axonal peripheral neuropathy in the lower limbs, dorsal column involvement and inflammatory myopathy were found by neurophysiology studies. There are 14 similar cases reported in Japan and Canada, but to our knowledge these are the first documented cases of HSCS in the tropics. A cerebellar syndrome constitutes another form of presentation of HTLV-I/II infection of the nervous system.
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PMID:Spinocerebellar syndrome in patients infected with human T-lymphotropic virus types I and II (HTLV-I/HTLV-II): report of 3 cases from Panama. 1087 59

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is characterized by spastic paraparesis in the lower extremities, and urinary disturbance. HAM/TSP has also been less frequently associated with cerebellar syndromes and nystagmus. We report a case of HAM/TSP presenting with cerebellar ataxia and nystagmus. The patient was a 73-year-old woman who was born in southern Japan. At age 41, she developed pain and spasticity in the bilateral lower limbs and gradually progressive gait disturbance. At age 57, she was diagnosed with HAM/TSP based on spastic paraparesis in the lower limbs, urinary disturbance and positive anti HTLV-I antibody in serum and cerebrospinal fluid. In June 2008, she was referred to our university and hospitalized for rehabilitation. Twenty days later, she experienced rotatory vertigo sensation. Magnetic resonance imaging revealed pontocerebellar atrophy. The patient presented with cerebellar signs in the upper limbs, gaze-evoked nystagmus in the sitting position and right-beating horizontal nystagmus in the supine and head-hanging positions. Electronystagmography (ENG) showed horizontal saccadic overshoot dysmetria and horizontal saccadic pursuit. Nystagmus is rare among the literature on HAM/TSP. ENG is helpful to evaluate and confirm the cerebellar syndromes of HAM/TSP.
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PMID:A case report of HTLV-I associated myelopathy presenting with cerebellar ataxia and nystagmus. 2103 92