Two cases of HTLV-I associated myelopathy (HAM) after cardiac operation

Sixty-one and 51-year-old males had progressive walk disturbance after cardiac operations at the intervals of 1 and 2 years. They had received blood transfusion at their operations. Physical examination revealed spastic paraparesis, sensory disturbance of the lower and rectobladder disorder. High titers of anti-Human T-lymphotrophic Virus type-I (HTLV-I) antibody were found in sera and CNF in both cases. They were diagnosed as HTLV-I associated myelopathy (HAM). Because they lived in Aichi Prefecture where the virus is non-epidemic, they were possibly infected through the blood transfusion at their operations. For prevention of HAM, the anti-HTLV-I antibody of all donor blood should be checked before transfusion.     

Two cases of neurogenic bladder due to HTLV-1 associated myelopathy (HAM)

Case-1 (24-year-old female) had complained of slowly progressive urinary incontinence (since 14 years old) and gait disturbance (since 18 years old). A marked pyramidal disorder was observed, and anti-HTLV-1 antibody (1:640) was present in her peripheral blood. She was diagnosed as having HTLV-1 associated myelopathy (HAM). Repeated urodynamic studies (UDS) revealed exacerbation of overactive bladder and detrusor-sphincter dyssynergia (DSD) with the progress of the disease. Case-2 (48-year-old male) had complained of gait disturbance (since 32 years old) and progressive urinary hesitancy (since 46 years old). Physical examination revealed a marked pyramidal disorder. Anti-HTLV-1 antibody (1:200) and ATL-like cells were present in his peripheral blood. He was diagnosed as having HAM. The voiding cystourethrography demonstrated an abnormal change of the bladder wall. UDS revealed overactive bladder and marked DSD. Medications based on adrenocortical steroids and urological cares have improved urinary disturbance, in both cases.     

Disability and determinants of gait performance in tropical spastic paraparesis/HTLV-I associated myelopathy (HAM/TSP)

STUDY DESIGN: Cross-sectional. OBJECTIVES: The aim of this survey is to describe the disability profile in a group of tropical spastic paraparesis/HTLV-I-associated myelopathy patients, identifying the requirements for community ambulation. SETTING: Tertiary care unit, Rio de Janeiro, Brazil. METHODS: Seventy-two patients were assessed (49 female and 23 male), referred by tertiary care centers, when a clinical protocol was applied. RESULTS: The sample had an average age of 40 years and an average of 137 months of duration of the disease. The most prevalent aspects of disability found were in gait and sphincter control areas. A total of 72% of the patients were community ambulators and 17% were restricted to wheel chair. Age, strength and low-back pain interfere in activities of daily living (P<0.05). A positive correlation was found between community ambulation and the knee extensors (r=0.80) and ankle plantar flexors (r=0.74). Strength, age, low-back pain, duration of disease, asymmetric onset of the symptoms and spasticity interfered in the ability to walk (P<0.05). A rehabilitation program was proposed focusing on modifiable factors that affect disability level. CONCLUSION: It was possible to describe the profile of disability in this group of patients, identifying the requirements to the community ambulation.     

Bladder involvement in HTLV-I associated myelopathy

The HTLV-I infection was endemic in south western Kyushu. This human T-lymphotropic virus type I may cause HTLV-I associated myelopathy (HAM), a neurological disease characterized by a spastic paraparesis. And one of the minor diagnostic features of HAM is the presence of cystorectal disturbance. We experienced 35 HAM patients with a neurogenic bladder. A gradually progressive contracted bladder was observed in 3 of them. The main pathological finding in these patients was submucosal infiltration of lymphocytes. These findings suggest that immunologic mechanisms account for the development of bladder lesions.     

Neurogenic bladder in patients with cervical compressive myelopathy

We examined the urinary disturbances in 56 consecutive patients with cervical compressive myelopathy using the latest International Continence Society classification. Of the 56 patients with cervical compressive myelopathy, 29 (52%) had some urinary subjective complaints, whereas the remaining 27 (48%) had none. Urologic examination indicated that 8 of these 29 (28%) patients with urinary complaints had urologic disorders other than neurogenic bladder. Of the remaining 21 patients, only 6 (25%) were judged to have neurogenic bladder on urodynamic study. Urodynamic study may be of limited value in diagnosing urinary disturbance in cervical myelopathy. Further, four cases (83%) showed underactive bladder activity in voiding phase, and only one case (17%) showed overactive bladder activity in filling phase. These results were contrary to those of previous studies indicating that cervical compressive myelopathy is associated with overactive bladder activity in filling phase. There were no significant differences in motor or sensory Japanese Orthopedic Association scores between the patients with and without urinary complaints. However, the patients with urinary complaints had significantly longer durations of myelopathy and delayed motor evoked potential latencies than those without urinary complaints. After surgery, 19 of the 21 (90%) patients with urinary complaints showed recovery from urinary disturbance. Operations in patients with cervical myelopathy were also effective against urinary disturbance. Urinary complaints may be an indication for surgical treatment despite the results of urodynamic study.     

Osteoporosis in HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP)

Human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) has been associated with changes in extracellular matrix of neural tissue. HTLV-I infection has multiple other systemic effects. Extracellular matrix is important for bone mineral deposition. We examined bone mineral density (BMD) in patients with HAM/TSP. BMD was assessed by ultrasonographic calcaneous densitometry in 24 patients (7 males, 17 females) with HAM/TPS, and 23 healthy HTLV-I-seronegative controls matched by age and sex. Patients with HAM/TPS had a mean BMD T-score of -3.07 +/- 0.64 in males and -2.93 +/- 0.69 in females. Control patients revealed a T-score of -0.77 +/- 1.31 in males and -1.17 +/- 1.08 females. The difference in T-score between HAM/TSP patients and control groups is significant (P < 0.001). Of HAM/TPS patients, 7 of 24 (29.2%) had osteopenia (T-score between -1 and -2.5) and 17 of 24 (70.8%) were diagnosed with osteoporosis (T < -2.5). Respective figures for control patients were 10 of 23 (43.5%) with a normal T-score, 11 of 23 (47.8%) with osteopenia, and 2 of 23 (8.7%) with osteoporosis. After adjustment for age and sex, odds ratio of osteoporosis for HAM/TSP patients was 31.52 (95% confidence interval, 5.07 to 195.88). No correlation was found in HAM/TSP patients between T-score and age, menstrual status, gait functionality, or years of evolution of HAM/TSP. HAM/TSP patients have a significantly diminished BMD of the calcaneous that appears not to be explained by paresis, age, years of disease, menstrual status; may be the result of systemic alterations due to HTLV-1 infection.     

Neurogenic bladder in acquired immune deficiency syndrome (AIDS).

This is the first report of incidence of neurogenic bladder in patients with acquired immune deficiency syndrome (AIDS) and emphasizes that it is a significant cause of various voiding dysfunctions in these patients. Neurologic disease occurs in about one third of patients with AIDS. Both central and peripheral nervous systems may be involved. Urodynamic evaluation is necessary for assessment and management of neurogenic voiding disorders in this group of patients.     

Neurogenic bladder in neuroleptic malignant syndrome

Urinary retention or incontinence is not an infrequent clinical finding in patients with neuroleptic malignant syndrome. We studied the pathophysiology of this voiding disorder by urodynamic testing. It revealed involuntary bladder contraction and rigidity of external sphincter (dyskinesia). These findings are analogous to those in Parkinson disease patients and support the dopamine deficiency theory as the cause of neuroleptic malignant syndrome.     

Neurogenic bladder in normal pressure hydrocephalus

Five patients with normal pressure hydrocephalus who had urinary incotinence were found to have neurogenic bladders by cystometry. The bladder disturbance in this disorder should be considered the result of a specific defect of brain function and not just an artifact of gait disturbance or of dementia.     

Overactive bladder in cervical spondylotic myelopathy

Background

Overactive bladder (OAB) is a new disease concept defined by the International Continence Society in 2002. There have been no reports of OAB among patients with cervical spondylotic myelopathy assessed on the basis of symptom questionnaires.

Methods

One-hundred-and-six patients diagnosed with cervical spondylotic myelopathy and treated by use of laminoplasty were examined. The patients were classified into two groups, those identified as having OAB (OAB group) and those identified as not having OAB (non-OAB group), by use of the Overactive Bladder Symptom Score collected before and 1 year after surgery. The clinical results for the two groups were assessed. OAB symptom prevalence and post-operative symptom improvement were investigated 1 year postoperatively.

Results

Of the 106 patients, 50 were identified as having OAB (symptom prevalence 47.2 %). Of these 50 patients, symptom improvement was observed for only 14 (28 %) 1 year after surgery. For both groups good improvement on the basis of the Japanese Orthopedic Association score was observed 1 year postoperatively, but there were no significant differences between them.

Conclusions

Post-operative improvement of OAB symptoms in cervical spondylotic myelopathy patients was low, which indicated that OAB was most frequently attributable to non-neurogenic and idiopathic, but not neurogenic, causes. It is considered necessary to tell patients with cervical spondylotic myelopathy that the possibility of post-operative OAB symptom improvement is not high when the explanation for informed consent is given before the operation.     

HTLV-I viral-associated myelopathy after blood transfusion in a multiple trauma patient

This may be the first documented case in the United States and in the orthopedic literature of transfusion-transmitted human T-cell leukemia virus Type I (HTLV-I)-associated myelopathy (HAM). Progressive myelopathy occurred in a 58-year-old white man with serologic and molecular evidence of HTLV-I infection after multiple trauma and subsequent transfusion with multiple units of banked blood products. Symptoms of myelopathy occurred 15 months after the transfusions. Myelopathy from HTLV-I infection simulates a disorder of orthopedic interest. Physicians should be aware of the symptoms of HAM and unexplained myelopathy.