Spinocerebellar syndrome in patients infected with human T-lymphotropic virus types I and II (HTLV-I/HTLV-II):report of 3 cases from Panama

Abstract

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.

Neurologic abnormalities in HTLV-I– and HTLV-II–infected individuals without overt myelopathy

“…[20][21][22][23] A spinocerebellar syndrome has also been documented in a few case reports of HTLV-I-and -IIinfected patients. 24 A better understanding of the neurologic abnormalities associated with HTLV infection is important for the clinical care of infected patients. The etiology and pathogenesis of these abnormalities are poorly defined, and it is unclear whether they are a precursor to the development of HAM or part of a broader spectrum of HTLV-associated neurologic morbidity.…”

Pearls & Oy-sters: “Not multiple sclerosis” and the changing face of HTLV-1

“…3 On extensive literature search, we found a case report of a Japanese woman with HTLV-1 presenting with downbeat nystagmus and cerebellar vermian atrophy. 4 Another report from Scandinavia discusses three patients with HTLV-1-associated spinocerebellar syndrome.…”

“…4 Another report from Scandinavia discusses three patients with HTLV-1-associated spinocerebellar syndrome. 3 Overall, there have been nearly 14 cases of HTLV-associated cerebellar involvement reported from Japan and Canada.…”

“…The patient tested positive for HTLV-1 and in distinction to other cases associated with HTLV-1 infection, this patient lived in the United States and presented with a syndrome characterized by progressive episodes of vertigo and nystagmus, without tremor, dysmetria, or cerebellar atrophy by MRI. [2][3][4][5] Classically, patients infected with HTLV-1 develop HAM/TSP, a slowly progressive myelopathy characterized by spastic paraparesis, hyperreflexia, and early bladder symptoms. 5,[6][7][8][9] Recent data suggest that clinicians should broaden their definition of HTLV-1-associated neurologic disease.…”

HTLV-2 infection in injection drug users in King County, Washington

“…In addition, HTLV-2 can produce a neurologic syndrome similar to HTLV-1-associated myelopathy/ tropical spastic paraparesis (HAM/TSP), with spasticity in the lower limbs, leg weakness, and bladder dysfunction. Unlike HTLV-1 infection, HTLV-2-associated myelopathy commonly also includes peripheral neuropathy and ataxia [12][13][14]. Neurologic disease in patients with HTLV-2 infection is much less common than in patients infected with HTLV-1 or HIV [10].…”