HTLV-I associated myelopathy with Hashimoto's thyroiditis--a report of two cases

We reported two cases of HTLV-I associated myelopathy with Hashimoto's thyroiditis. Case 1 is a 47-year-old female, who has had Hashimoto's thyroiditis since the age of 29. At the age of 38, she first noticed difficulty in walking which progressed gradually. At the time of examination, she showed a spastic gait, increased deep tendon reflexes in four extremities, hypesthesia of the feet, mild bowel and bladder disturbances. Anti-HTLV-I antibody was increased to the titer of 16,834 in the serum and of 32 in the cerebrospinal fluid by PA method. Hypothyroidism was present and the titers of microsome test and thyroid test were elevated to 102,400. Anti-T4, and anti-T3, and anti-thyroglobulin antibodies were found. Ultrasonography of the thyroid revealed its enlargement and decreased echo signals. Thyroid scintigram showed inhomogeneous 123I uptake and some defective areas of the uptake. Histological examination of the biopsy specimen of the thyroid demonstrated marked lymphocytic infiltration, germinal centers, hypertrophy of follicular epithelium, and interstitial fibrosis, which were consistent with Hashimoto's thyroiditis. Case 2 is a 60-year-old female who has had slowly progressive gait disturbance since her childhood. She became unable to walk at the age of 35. At the time of examination, she had a spastic gait, increased deep tendon reflexes bilaterally, and positive Babinski signs. Anti-HTLV-I antibodies were increased to a titer of 8,192 in the serum and of 32 in the cerebrospinal fluid. Thyroid function was normal. However, anti-thyroid antibodies were positive and histology of the thyroid showed lymphocytic thyroiditis, which were consistent with mild grade of Hashimoto's thyroiditis.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of HTLV-I associated myelopathy with abnormal patchy MRI lesions in the spinal cord]

We report a case of HTLV-I associated myelopathy (HAM) with a spinal cord MRI showing abnormal multifocal and patchy lesions. A 50-year-old woman suffering from progressive paraparesis was admitted to our hospital. HTLV-I antibodies in the serum and CSF were positive, and a diagnosis of HAM was made. Her T2 weighted spinal cord MRI showed scattered areas of high signal intensity from the cervical to the thoracic cord. The lesions were enhanced with gadolinium-DTPA on T1 weighted imaging. Atrophy of the thoracic cord has been reported in many patients with HAM. In rare cases, T2 weighed thoracic cord MRI showed diffuse high signal intensity. The pattern of high signal intensity in our case, however, was multifocal and patchy, thus differing from the findings of previous reports. And we believe this is the first such report. This case suggests that the MRI of HAM patient may show multifocal and scattered lesions in the spinal cord.     

HTLV-I associated myelopathy in Brazil: a preliminary report

In this preliminary report the authors present the finding of a high prevalence (37.5%) of seropositivity of antibodies to HTLV-I tested by Western blotting in a sample of 16 Brazilian patients with chronic myelopathies of undetermined origin.     

HTLV-I associated myelopathy (HAM) and sarcoid myopathy]

A 65-year-old house-wife developed dirty erythematous rash on her face in April, 1989. Almost simultaneously, she complained of muscle soreness and weakness on both lower extremities. Pathological findings of the skin biopsy at that time was consistent with those of sarcoidosis with moderate inflammatory cell infiltration. In December, 1989, when she was admitted to our hospital, her lower extremities were paretic with marked spasticity, and mild bladder dysfunction was noted. HTLV-I antibody titers in serum and cerebrospinal fluid were significantly elevated. Biopsied limb skeletal muscle revealed the findings of the sarcoid myopathy with small inflammatory cell infiltration in endomysium. HLA haplotypes showed A24, B7, BW61, CW7, CW8, DR1 and DR4 which show relatively common types of those in HAM. Corticosteroid treatments including the methylprednisolone pulse therapy healed the skin lesion, but did not improve her neurological signs. Paraplegia and urinary disturbance were progressive. It is concluded that the inflammatory sarcoid myopathy with HAM in this patient may be caused by a common abnormal immunological background.     

Pathological observations in HTLV-I associated myelopathy

The following are the clinical and autopsy findings in a 63-year-old woman with myelopathy associated with the human T-cell lymphotropic virus Type I (HTLV-I). HTLV-I antibody was positive in both the serum and cerebrospinal fluid (CSF). In the lower thoracic region, demyelination and the loss of axons were accompanied by a proliferation of astrocytes, and gliosis was found in the lateral columns. Perivascular and parenchymal infiltrations of macrophages, lymphocytes, and plasma cells were also observed, but neither viral inclusion bodies nor atypical lymphocytes were found.     

Holoprosencephaly (lobar form) associated with bilateral vocal cord palsy.

There have been many causes associated with bilateral vocal cord palsy, both congenital and perinatal. Until now, the main congenital causes of bilateral vocal cord palsy have been associated with meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. We report a patient with bilateral vocal cord palsy associated with the lobar form of holoprosencephaly. In this case, bilateral vocal cord palsy was caused by the disruption of the cortical laryngeal motoneurons. Neonatal stridor must be carefully evaluated by the neonatologist to exclude severe cerebral anomalies.     

Upper airway obstruction with bilateral vocal cord paralysis secondary to ischemic stroke: report of two cases]

We present herein two patients with bilateral vocal cord paralysis that occurred during the subacute phase of brain infarction. Patients were a 73-year-old man and an 82-year-old woman who suffered from infarction of the basilar artery and the right middle cerebral artery, respectively. The former was diagnosed as atherothrombotic infarction, but the patient experienced repeated aggravation. The latter was diagnosed as cardioembolic infarction. After both patients took clopidogrel and warfarin for the secondary prevention of stroke, upper airway obstruction developed at Day 29 and Day 19, respectively. Vocal cords in Case 1 did not show any movement on laryngoscopy, and were fixed together nearly closed. In Case 2, vocal cords were again almost fixed together. Bilateral vocal cord paralysis is a common complication of cervical operations, but is rare after ischemic stroke. As patients who have suffered from bilateral vocal cord paralysis are often facing death, we must be careful of wheezing with ischemic stroke.     

HTLV-I associated myelopathy (HAM) complicated with chronic polymyositis

The patient was a 49-year-old woman. Since her age of 42, she noticed a proximal weakness of both legs. She also experienced pedal paresthesia and urinary frequency. Physical examination disclosed a diffuse goiter and bilateral Babinski sign. Results of EMG and muscle pathology were compatible with the diagnosis of polymyositis. Treatment with prednisolone improved muscle weakness, urinary difficulties and struma. 7 years later, HTLV-I antibody happened to be strongly positive both in serum and CSF. Then most of her neurological problems were attributed to HAM. However, recent studies of re-biopsy muscle specimens disclosed scattered necrotic fibers, phagocytosis and endomysial or perivascular infiltration of inflammatory cells. These infiltrating cells were classed mostly as helper/inducer T cells. These observations suggested the coexistence of polymyositis in the present case as well. So far, the combination of HAM and polymyositis has not been reported. It seems important to decide if HTLV-I could induce chronic polymyositis as in the case of HIV infections.     

Multimodality evoked potentials in HTLV-I associated myelopathy.

Multimodality evoked potentials (EPs) consisting of somatosensory EPs (SEPs), visual EPs (VEPs) and brainstem auditory EPs (BAEPs) were studied in 16 cases with HTLV-I associated myelopathy (HAM). Median nerve SEPs were normal in all cases. In posterior tibial nerve SEPs, the potential recorded at the 12th thoracic spinal process was normal in every case but cortical components were significantly prolonged in 10 cases, although five of these showed no sensory impairment. BAEPs were normal in every case whose hearing was intact, but VEPs were abnormal in two cases whose visual acuities were normal. The present results in HAM indicate predominant lesion in the thoracic cord, and might also suggest some subclinical lesion in the visual pathway.     

A case of juvenile Parkinson disease with vocal cord abductor paralysis in the course of malignant syndrome]

We report a 60-year-old woman with juvenile Parkinson disease (PD) with vocal cord abductor paralysis (VCAP). She had suffered from juvenile PD for 30 years. She was admitted in February 1998 to our clinical unit, because of malignant syndrome induced by dehydration. Neurological examination revealed disturbance of consciousness, hand tremor, dyskinesia of the trunk and all extremities, and rigidity. Laboratory examinations disclosed leukocytosis, renal dysfunction, hypermyoglobinemia, and elevation of the serum creatine kinase. Six days after admission, dyspnea and inspiratory stridor were noted, and the respiratory distress worsened. Endoscopy of the upper airways revealed that the vocal cord was in the midline or paramedian position. There are some cases of PD with VCAP, but such a case is very rare in Japan. We discussed the pathogenic mechanisms of these conditions, and speculated that VCAP was associated with malignant syndrome in our case.     

A case of anti-GM1 antibody positive HTLV-I associated myelopathy (HAM) with polyneuropathy]

We report a case of HTLV-I associated myelopathy (HAM) with polyneuropathy. A 59-year old man suffering from progressive paraparesis associated with subclinical polyneuropathy was admitted to our hospital. HTLV-I antibodies in the serum and CSF were positive, and a diagnosis of HAM was made. His laboratory investigation revealed elevated serum IgG and IgM anti GM-1 antibodies. The nerve conduction study showed a mild reduction in motor and sensory conduction velocity in all extremities. A sural nerve biopsy revealed active demyelination and globule-like changes, which are specific for HAM neuropathy. Anti-GM1 antibodies are frequently present in autoimmune motor neuropathy. They are thought to inflict a damage on both the myelin and axons of the peripheral nerves. Ours is believed to be the first case of HAM associated with anti-GM1 antibodies, although polyneuropathy is often associated with HAM. While it is not clear whether the lesion observed in HAM neuropathy results from the direct cytopathic effect of the virus or from the immune response, some immune-mediated reactions are thought to play an important role. This case suggests that a case of HAM with polyneuropathy should be examined for the presence of the anti-ganglioside antibodies. More investigations are needed to fully understand the mechanism of the HAM neuropathy.     

Medulla and upper cervical cord compression by bilateral vertebral artery presented with myelopathy and drop attack: case report]

A 51-year-old man had suffered from attacks of quadri-paresis and unconsciousness for previous three years prior to presentation. Prior to admission, he had been received anticonvulsants, but his symptoms showed no improvement. Neurological examination revealed hyper-reflexia of his left lower extremity and moderate decrease of sense of pain, temperature, and tactile sensation in his left extremities and trunk, while vibratory sensation was normal. Magnetic resonance(MR) imaging revealed a flow-void area in the craniocervical junction and marked narrowing of the medulla oblongata and upper cervical cord by compression of the vertebral arteries(VA). CT myelography also showed the compression and narrowing of the spinal cord. Vertebral angiography demonstrated symmetrical running course of the arteries, which curved medially at the level of craniocervical junction. Suboccipital craniectomy and C1 and upper half of C2 laminectomies were performed. After dural opening, the ventrolateral aspects of the lower medulla oblongata and the upper cervical cord were found to be compressed by the VA. The arteries were retracted dorsolaterally by GORE-TEX tapes so as to decompress the medulla oblongata and cervical cord, and the tapes were anchored to the residual part of C1 posterior arch. Postoperative MR imaging and CT myelography showed complete decompression, and the patient was relieved of his previous neurological symptoms.     

A case of HTLV-I associated myelopathy presenting with cerebellar signs as initial and principal manifestations]

We reported a 75-year-old woman with HTLV-I associated myelopathy (HAM) presenting with cerebellar signs. She was admitted to our hospital because of walking unsteadiness, which initially appeared 3 years previously with gradual worsening. Neurological examination revealed limb and truncal ataxia, cerebellar type dysfunction of eye movement, pyramidal sign, diminished vibration sense and neurogenic bladder. Anti HTLV-I antibody titers in serum and CSF were markedly elevated. MRI revealed abnormal signals in cerebral white matter, mild cerebellar atrophy and thoracic cord atrophy. Cerebellar signs and symptoms were initial and main neurological manifestations in this patient, which were improved by steroid therapy. We considered this case was unique among HAM, because cerebellum was considered her main lesions.     

Expression of HTLV-I antigen in cultured peripheral blood mononuclear cells from patients with HTLV-I associated myelopathy

The HTLV-I antigen (Ag) was detected in short-term cultured peripheral blood mononuclear cells (PBMNC) from 44% of patients with HTLV-I-associated myelopathy (HAM), using the indirect immunofluorescence method. The HTLV-I Ag-positive cells accounted for less than 4% of cultured PBMNC in all but one case. The kinetics and mechanism of HTLV-I Ag expression in cultured cells were these studied in this individual with about 20% positive cells. HTLV-I Ag was 0.3% at 6 h after the culture and the number of positive cells increased to 9.3% at 48 h. The sera from HAM patients had a suppressive effect on the expression of HTLV-I Ag in the cultured cells. This suppression was more potent in sera from patients with a high than with low antibody titer. There were no correlations between the HTLV-I Ag expression in cultured cells and the various clinical and laboratory findings, in each case.     

HTLV-I associated myelopathy in Salvador (northeastern Brazil).

Recent studies of tropical spastic paraparesis have confirmed the existence of human T-cell leukemia virus type-1 (HTLV-I) in several tropical areas of the world. In order to determine the role of HTLV-I as an etiologic agent of myelopathies in Salvador, we conducted a clinical and serological study in 43 patients with non-traumatic and non-tumoral myelopathies. We found 9 patients with HTLV-I associated myelopathy (HAM) which points to a new endemic area of HAM.     

Electroneuromyography and somatosensory evoked potentials in HTLV-I associated myelopathy

The objective of the present study was to correlate electroneuromyography (ENMG) and evoked potentials findings with clinical aspects and intrathecal synthesis of HTLV-I antibodies production on HTLV-I myelopathy (HAM). Patients were seropositive for HTLV-I by different assays and seronegative for HIV and VDRL. They had no other causes of myelopathy and peripheral neuropathy. Peripheral neuropathy was established in 34.3% of the cases by ENMG. Peripheral neuropathy was mostly asymmetric (82%), sensory motor (90%), axonal (54.5%) or of a mixed type (45.4%). In 63.6% of these cases related symptoms were observed. ABR was abnormal in one patient and the PRVEP in 28.5%, who were symptom-free. The SEP was abnormal in 85.7% of the cases, half of them presenting clinical complaints. In only 14% of the individuals with clinical manifestations, SEP was normal. In 28% of patients with abnormal SEP the ENMG disclosed a peripheral neuropathy.