Akihisa Matsumoto

Spinal Magnetic Resonance Image alterations in human T-lymphotropic virus type I-associated myelopathy patients before and after immunomodulating treatments.

Sirs: Previous reports have been that most of the spinal cord lesions of Human T-lymphotropic virus type I associated myelopathy (HAM) were atrophy induced by the chronic inflammatory processes, and that these changes seldom showed alterations in MRI signal intensity [1–3]. In contrast, we present here two cases of HAM patients in whom the spinal cord showed moderate swelling both with gadolinium-DTPA enhancement and faint high T2 signal intensities, and in whom these alterations were partially resolved after interferon-alpha and steroid treatments. Case 1: A 66-year-old woman noticed paresthesia in her left arm and walking difficulty about eight months prior to admission. Suspected of having cervical and thoracic myelopathy, she underwent decompression of the cervical and thoracic spine at another hospital. Her neurological symptoms did not significantly improve, and in fact, there was gradual worsening. Neurological examination revealed marked spasticity of the lower extremities, and slight spasticity of the upper limbs. Deep tendon reflexes were hyperactive, and pathological reflexes were easily elicited. Serum antibody for HTLV-I was elevated to x8132, as was that of CSF x32. Additionally, Western blot analysis demonstrated P19 and p24 in the CSF. Protein in the CSF was elevated to 55 mg/dl, and IgG was 7.7 mg/dl (normal; 4 mg/dl >). No pleocytosis was seen (3/mm3). Both oligoclonal bands and MBP (myelin basic protein) were negative. On the basis of these findings, the patient was diagnosed as having HAM. Previous T2-weighted MRI performed prior to operation demonstrated high signal intensity in the patient’s cervical and thoracic cord (Fig. 1A,B). Moreover, the spinal cord showed faint Gdenhancement. Cranial MRI did not show white matter lesions, which are well known to be associated with HAM. As the initial treatment, we administered three million units of interferon-alpha intramuscularly daily for twenty-eight days. Following the interferon treatment, pulsed steroid therapy was employed, and oral steroid dose was gradually tapered. Then, the frequency of the tonic spasms in the lower extremities decreased. Intriguingly, MRI showed that T2 high signal intensity and gadolinium-DTPA enhancement of the spinal cord had almost disappeared (Fig. 1C,D). Case 2: A 51-year-old woman was transferred to our department because she had had walking difficulty and dull pain over both her lower extremities for about four months prior to admission. Neurologically, the spasticity of her extremities was prominent, and deep tendon reflexes were markedly hyperactive. Pathological reflexes were easily elicited. She also showed a markedly unsteady gait. Her serum anti-HTLV-I antibodies were highly elevated (x16384). Since her CSF examination had also revealed a high titer of HTLV-I antibody (x1024), and Western blotting had demonstrated p19 and p24 proteins, we concluded that she was suffering from HAM. MRI demonstrated cervical and thoracic cord swelling with faint T2 high signal intensities (Fig. 2A,B,C). Additionally, gadolinium-DTPA enhancement in the cervical cord revealed high signal areas in the lateral columns, which resembled “owl eyes” (Fig. 2D). Cranial MRI was regarded as normal. The spinal fluid showed slightly elevated protein 46 mg/dl and mild pleocytosis (33/mm3). IgG in the CSF was 6.7 mg/dl (normal; 4.0 mg/dl >). Neither oligoclonal bands nor MBP were found. The patient was initially administered steroid (1 mg/kg), and the dose was gradually tapered. In addition to the steroid treatment, interferon-alpha was administered every day for twenty-eight days. Her spasticity appeared to decrease, DTR became slightly hyperactive, and bilateral pathological reflexes showed weakly positive. Her walking speed increased, and a steadier gait was seen. MRI revealed a less swollen spinal cord, and the previously observed T2 high signal in the cervical cord had disappeared (Fig. 2E). Despite the improvement in cord swelling, T2 high signal intensities within the thoracic cord remained (Fig. 2F). In spite of intensive investigation including auto-antibody analysis, systemic CT and scintigrams, no other systemic disorders which could generate myelopathies were found in either case. However, we cannot completely exclude the possibility that protruding cervical disks at C5/6 and C6/7 in case1 and LETTER TO THE EDITORS

Elbow flexion response as another primitive reflex

Abstract In daily clinical practice we noticed that patients with intellectual impairment spontaneously flex the elbow within a few seconds of the forearm being manipulated during routine examination of spasticity of the muscles in the upper extremities. We termed this phenomenon elbow flexion response (EFR), and prospectively studied it in 229 patients who underwent in‐hospital rehabilitation following brain damage. Evaluation of each patient included EFR, patient profile, ability to communicate, scores on three parameters from various intelligence tests, scores on seven parameters testing primitive reflexes, and scores on three parameters describing personality. We investigated for relationships among these parameters. Consequently, although EFR rarely have a statistical association with the varied profiles of patients, patients with bilateral lesion or bilateral paresis demonstrated significantly more marked EFR than those with unilateral lesion or unilateral paresis. Patients with involvement of the frontal lobe showed significantly more marked EFR than those without damage in this area. Elbow flexion responses occurred significantly more frequently in relation with lower scores on intelligence and occurred with significantly higher frequency in conjunction with the more marked appearance of conventional primitive reflexes. Therefore, we conclude that EFR have a strong association with intelligence and with the existence of frontal lobe lesion, and their mode of clinical presentation parallels that of primitive reflexes particularly that of the grasp reflex. We propose that EFR could be referred as a variation of the grasp reflex occurring in the more proximal or axial part of the body.

Femoral neuropathy induced by a low-grade myofibroblastic sarcoma of the groin.

Sirs: Femoral neuropathy is an uncommon disorder [4] and has sometimes been found to be associated with a pelvic hematoma due to anticoagulation therapy [10], the lithotomy position [1], pelvic surgery [7], renal transplantation [8] and radiation injury [6]. Here we report a case of femoral neuropathy induced by a low-grade myofibroblastic sarcoma (LGMS) of the groin, which is a recently established pathological entity [3], and which explains the characteristic radiological findings. A 64-year-old woman noticed numbness of the inner surface of the left leg, which was gradually spreading. An orthopedist made a diagnosis of lumbar spinal disk disease and prescribed a muscle relaxant. In spite of this, the patient’s left leg became gradually weaker and the sensory disturbance worsened. She then began to drag her left leg while walking and was referred to our department 2 months later. A neurological examination revealed weakness and muscle atrophy in her left quadriceps muscle. The left knee jerk reflex was decreased and numbness in the saphenous nerve distribution was found. There was no evidence of central nervous system involvement. On the basis of these clinical findings, the function of the left femoral nerve was considered to be impaired. The motor conduction velocity between above and below the inguinal ligament was estimated to be 64m/s for the right femoral nerve, and 33m/s for the left femoral nerve. Electric stimulation proximal to the inguinal ligament demonstrated that the amplitude of the motor action potential in the rectus femoris muscles was markedly reduced on the left side (8.9 mV on the right and 1.5 mV on the left). When the left saphenous nerve was stimulated antidromically, the sensory action potential decreased to 3.9 μV (7.3 μV in the right saphenous nerve), though nerve conduction was not impaired. This implied the presence of axonal damage in the distal part of the femoral nerve. Other nerve conduction studies showed no evidence of generalized peripheral neuropathy or lumbosacral plexopathy. Needle electromyographic (EMG) evaluation could only be performed for the left rectus femoris muscle, which indicated positive sharp waves and fibrillation potentials at rest. The patient declined further EMG investigation owing to the pain. CT revealed an ill-defined mass lesion in the left groin (Fig. 1a). This was a tumor that appeared to be infiltrating into the retroperitoneal area and surrounding muscles. It also showed heterogeneous enhancement and involved the left femoral nerve (Figs. 1a and b). Compared with the right femoral nerve, the left femoral nerve appeared to be enlarged and probable gadolinium enhancement was observed close to the tumor (Fig. 2). Short-tau inversion recovery (STIR) MRI of the lower leg revealed homogeneous high-intensity signal changes in the left denervated quadriceps muscle and sartorius muscle supplied by the femoral nerve (Fig. 3) [9]. In order to make a precise diagnosis, LETTER TO THE EDITORS

Primary CD30/Ki-1 Positive Anaplastic Large Cell Lymphoma of the Central Nervous System Occurring in a Patient with a Seventeen-Year History of Essential Thrombocythemia

We present a rare case of central nervous system lymphoma that occurred in a patient who had essential thrombocythemia for 17 years. MRI examinations disclosed multiple ring-enhanced lesions that had shown bilateral spreading in the different period. Pathological examinations confirmed CD30/Ki-1-positive ALK negative anaplastic large cell lymphoma. The possible pathogenic mechanisms of this disease are discussed.

A Cross-Sectional Study on Socioeconomic Systems Supporting Outpatients With Parkinson's Disease in Japan.

OBJECTIVES We conducted a cross-sectional study to evaluate the socioeconomic systems supporting outpatients with Parkinsons disease (PD) in Japan. METHODS The study was performed in 2013 at two private hospitals and one clinic in Hokkaido Prefecture, Japan. A survey was conducted with 248 consecutive PD patients, and the data from 237 PD outpatients were analyzed after excluding 11 patients who did not meet inclusion criteria. Monthly medical and transportation payments as a PD outpatient were selected as outcome variables, and their association with various explanatory variables, such as utilization of support systems for PD outpatients, were evaluated using logistic regression model analysis. RESULTS After controlling for potential confounding variables, the utilization of the system providing financial aid for treatment for patients with intractable disease was significantly inversely associated with monthly medical payment among PD outpatients (OR 0.46; 95% CI, 0.22-0.95). Experience of hospital admission for PD treatment was significantly positively associated with monthly transportation payment (OR 4.74; 95% CI, 2.18-10.32). Monthly medical payment was also significantly positively associated with monthly transportation payment (OR 4.01; 95% CI, 2.23-7.51). CONCLUSIONS Use of Japanese public financial support systems may be associated with reductions in medical payments for PD outpatients. However, those systems may not have supported transportation payments, and higher transportation payments may be associated with an increased risk of hospitalization.

Analysis of resources assisting in coping with swallowing difficulties for patients with Parkinson’s disease: a cross-sectional study

BackgroundMalnutrition induced by swallowing difficulties (SD) impairs the quality of life and gives rise to SD-related costs in Parkinson’s disease (PD) patients. With results of a swallowing difficulty questionnaire and data of resources specifically obtained such as SD-related costs, caregivers, and dietary therapies, this study is to suggest statistically supported ideas for improvements in arrangements for how participants cope with SD and maintain general well-being.MethodsWe interviewed 237 PD patients. The SD-related costs involved those incurred by the provision of dietary modifications, care oriented foods, alternatives, and supplements. Dietary therapies included rice porridge and commercially available care foods. The relationships between BMI (body mass index) and the severity of SD assumed in this paper as indicators for general well-being and as resources for coping with SD for PD patients were statistically analyzed.ResultsA lower BMI was found in participants eating porridge consistency rice (p = 0.003) and eating porridge rice is significantly related to the severity of SD (p < 0.0001) and PD (p = 0.002). The severity of SD increased with age and PD duration (p = 0.035, p = 0.0005). Outlays for dietary modifications are the lowest reported here (p < 0.004) but the number of participants using dietary modifications is the largest among the SD-related items (n = 58). Eating care foods were reported for 11 older participants (p < 0.0001), most female (10/11). No lower BMI was found in participants eating care foods when compared with participants eating ordinary foods. Dietary modifications were performed by caregivers (OR: 6.8, CI: 3.1-15.2, p < 0.0001) and were related to the presence of children (OR: 3.4, CI: 1.2-11.4. p = 0.024). Older participants commonly live with spouses and children.ConclusionsSevere SD is associated with higher costs of coping with SD. A lower BMI is associated with modified foods, mostly eaten to cope with SD. Presence of caregivers and other persons residing with the participants here are related to dietary modifications but not to care food-related costs. Care foods may be effective in preventing malnutrition although the number who are able to cover the added expenses is limited because of the higher prices and shortage of information on the usefulness of care foods.

An episode of cerebrospinal fluid leak syndrome involving a marathon runner

A 57-year-old marathon runner presented with orthostatic dizziness and headache, which appeared just after running a few kilometres and had persisted for 7 days. No other neurological abnormalities were found. On lumbar puncture, the initial pressure was zero. A cranial MRI with gadolinium showed a marked enhancement of the dura mater (figure 1, arrows). MR …