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Type 2 diabetes mellitus patients are immunocompromised, particularly under poorly controlled conditions, and thereby they could develop rare inflammatory diseases, such as spontaneous discitis, pyogenic psoas abscess, spinal epidural abscess and bacterial meningitis. Herein we report a pyogenic psoas abscess on the dorsal side, and bacterial meningitis and spinal epidural abscess on the ventral side, both of which were induced by spontaneous discitis in a patient with poorly controlled type 2 diabetes mellitus. This case was very rare and interesting, because we successfully treated various infections with antibiotics over a long period of time, complicated by hyperglycemic crises, although the patient suffered severe bone destruction and required rehabilitation for a long time. more...

Objective To achieve an accurate quantification in diabetic polyneuropathy (DPN), we developed a new electrophysiological index that we called the DPN index. The relationship between the DPN index and the neurological findings in diabetic patients was assessed. Methods The DPN index was calculated by the mean value of percentages of four parameters (tibial compound muscle action potential amplitude / F wave minimum latency, sural sensory nerve action potential amplitude / sensory nerve conduction velocity) against the mean normal values. Twenty healthy subjects were recruited as a control group. Patients A total of 348 diabetic patients who were hospitalized in our hospital during the period from December 2016 to August 2019 were retrospectively studied. The correlations between the DPN index and five neurological findings (subjective sensory symptoms, diminished or absent Achilles tendon reflex, impaired tactile and vibration sense, low coefficient of variation of R-R interval) were evaluated. Results The DPN index in healthy subjects was 129.3±32.7%. The DPN index in diabetic patients with one or more neurological findings was significantly lower than that in diabetic patients without any neurological findings (p more...

Background There are few reports of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-positive, unilaterally dominant cerebral cortical encephalitis onset with epilepsy. We present such a case in a young female patient with myelitis. Case presentation A 19-year-old female developed generalized tonic seizures lasting several minutes. She had a low-grade fever and headache without other clinical neurological abnormalities while at our hospital. Cerebrospinal fluid (CSF) showed mononuclear pleocytosis. Other laboratory tests indicated no apparent abnormalities. Unilateral meningeal hyperintensity was seen on T2 fluid-attenuated inversion recovery MRI with associated cortical swelling and gadolinium enhancement of the cortical layer. One thousand mg/day of levetiracetam and a 3-day course of intravenous methylprednisolone at 1000 mg/day were administered. Dysuria appeared on the twentieth day of illness, and spinal MRI revealed a longitudinally extensive cord lesion from C5 to L1 consistent with myelitis. Two cycles of a 3-day course of intravenous methylprednisolone at 1000 mg/day were administered, and all symptoms disappeared. We found the patient to be anti-MOG antibody-positive using serum and CSF (titer: serum 1:256; CSF 1:128). Conclusion Our report illustrates a unique case of anti-MOG presenting as new onset epilepsy secondary to unilaterally dominant meningoencephalitis preceding the onset of longitudinally extensive transverse myelitis. more...

Aim The underlying etiology of dropped head syndrome (DHS) in Parkinson's disease (PD) patients seems to be heterogeneous, that is neck dystonia, a drug-induced etiology or focal myopathy of the neck extensor muscles. Focal myopathy might be primary myositis or a stretched muscle injury. In Japan, several reports have argued that neck dystonia is the cause of DHS. There have been no reports to date that documented a focal myopathy of the neck extensor muscles. The aim of the present study was to investigate myopathic changes in the neck extensor muscles of PD patients with DHS by needle electromyography (EMG), and to evaluate the effect of steroid therapy. Methods We carried out needle EMG on 15 consecutive PD patients with DHS who visited our hospital between July 2009 and November 2011. The effects of prednisolone for the treatment of DHS were evaluated clinically and electrophysiologically. Results Needle EMG of the neck extensor muscles in 15 PD patients with DHS revealed fibrillation potentials, positive sharp waves and small amplitude, short duration motor unit potentials in 14 patients. EMG findings were indicative of focal myopathy in five patients, and were compatible with focal myopathy in nine patients. DHS improved in eight out of 11 patients given prednisolone 20 mg/day. Follow-up needle EMG after steroid therapy in four patients showed improvements in the myopathic changes. Conclusion We report that focal myopathy of the neck extensor muscles is common in Japanese PD patients with DHS. Our findings suggest that steroid therapy might improve DHS in some PD patients. more...

Neck dystonia is the most common cause of dropped head sign in parkinsonism. Isolated neck extensor myopathy, which is a rare condition, can also cause dropped head sign in parkinsonism, but no improvement has been achieved with immunosuppressive therapy. We report three cases of treatable neck extensor myopathy causing dropped head sign in patients with Parkinson's disease. Needle electromyography and magnetic resonance imaging suggested a restrictive active myopathy affecting neck extensor muscles. All cases responded dramatically to steroid therapy. Routine needle electromyography should be performed to explore treatable myopathy in Parkinson's disease. more...

We evaluated two patients who were considered as having complicated form of familial spastic paraplegia (FSP). Patient 1 was a 37-year-old male, having slowly progressive walking difficulty. Examination revealed dementia, nystagmus, slurred speech, spastic paraparesis and increased reflexes. His sensation was normal. Patient 2 was a 35-year-old male. Examination revealed dementia, dysarthria, spastic paraparesis, increased reflexes and slightly decreased vibration sense in the ankle. We examined the median and tibial short-latency somatosensory-evoked potentials (SEPs). In patient 1, N9o-P13/14o and P13/14o-N20o intervals were prolonged in the median SEPs. ‘N9o’ means N9 onset. N21–N30 and N30–P38 intervals were prolonged in tibial SEPs. In patient 2, P13/14o latency was normal, but N20 was lost in the median SEPs. N30 latency was normal, but P38 was lost in the tibial SEPs. The present results indicate defective transmission in the somatosensory pathway, especially in its intracranial portion, in patients with complicated form of FSP. This contrasts with findings in patients with pure form of FSP, who usually show defective transmission only in the thoracic spinal portion. SEPs showed abnormal findings, even in a patient without sensory disturbances. SEPs are useful for localizing the lesion sites and for differential diagnosis between the pure and complicated forms of FSP. more...

A 77-year-old man was admitted because of muscle weakness in both upper and lower extremities. Diabetes mellitus was diagnosed in 1988 and he had been treated by oral hypoglycemic agents. He had a common cold at the end of January, 1997. Muscle weakness appeared in the upper extremities, followed by the lower extremities at the end of February. No sensory disturbance or dysuria was recognized. Nerve conduction study revealed distally dominant demyelinating polyneuropathy. Guillain-Barré's syndrome was diagnosed and he recovered completely following immunological absorption therapy (IAT). However, he had quadriplegia again at the end of April. He was treated by IAT combined with corticosteroid and has shown no relapse. In June, 1997, gastric cancer was detected by upper gastrointestinal fiberscopy and subtotal gastrectomy was performed. Judging from this clinical course, this case seems to be chronic inflammatory demyelinating polyneuropathy (CIDP) with acute onset. Many kinds of causes often contribute to the pathogenesis of neuropathy in the elderly. So in cases of progression or worsening, we should consider such possibilities and it is necessary not to exclude treatable causes of neuropathy. more...

The purpose of this study was to assess the relationship between mental fatigue and event-related potentials (ERPs). Six healthy men (mean age: 22 years old) performed a simple calculation for 6 h. Auditory ERPs were recorded before and after the calculating task. The scores of subjective fatigue symptoms were significantly increased, and P300 latency of auditory ERPs was significantly prolonged by the calculating task (P more...

A 54-year-old man presented with tremor and unusual behavior. He was admitted two months later because of dementia and myoclonus. Periodic synchronous discharges were observed on the electroencephalogram. Based on these findings, we diagnosed the case as Creutzfeldt-Jakob disease. About two weeks after admission, decubitus, bowel dysfunction and hypohidrosis occurred. We observed various autonomic nervous system dysfunctions such as abnormal pupillary response to autonomic drugs, reduced coefficient of variation of R-R interval, and abnormal diurnal blood pressure variation. more...

Undifferentiated carcinomas of the colon are rare, especially macroscopic expansive polypoid type. Only few cases have been reported so far. Recently, we experienced such a rare case. A 71-year-old male patient came in the hospital because of some symptoms suggestive of intestinal obstruction. General diagnostic work-up, including colon biopsy, revealed a primary undifferentiated carcinoma of the sigmoid colon and radical operation was performed. Undifferentiated carcinomas of the colon and rectum would demand general diagnostic work-up in the differentiation from others such as carcinoid, malignant lymphoma, malignant melanoma, and metastasis of pulmonary carcinoma. In addition appropriate selection of antitumor drugs in terms of histological findings would be important in the adjuvant chemotherapy. more...

A 27-year-old woman presented with acute paresis after taking an oral polio vaccine (OPV). Deep tendon reflexes were preserved, needle electromyography showed no neurogenic changes, and there were no lesions on spine magnetic resonance imaging (MRI), suggesting that motor neurons of the spinal cord were not affected. Brain MRI showed abnormal lesions in the tegmentum of the upper pons, left cerebral peduncles, truncus of the corpus callosum, and right parietal lobe. Cerebrospinal fluid revealed mild pleocytosis. The most probable diagnosis was acute disseminated encephalomyelitis associated with OPV. more...

OBJECTIVE: The nerve conduction findings in interdigital neuropathy of the foot (IDN; Morton's neuroma) have rarely been reported. We analyzed the nerve conduction data in 23 patients with suspected IDN studied between 1982 and 2002. METHOD: Diagnosis of IDN was made on the basis of clinical features. All patients underwent routine nerve conduction studies and a near-nerve needle sensory nerve conduction study of the interdigital nerves by Oh's method in the symptomatic foot. RESULTS:Of the 23 patients, the diagnosis of definite IDN was made in 13 cases and of possible NDN in the others cases. Nineteen were females. Twenty two patients had only one nerve affected. One patient had two nerves affected. The III-IV interdigital nerve was affected in 17 cases and the II-III interdigital nerve in 7 cases. The near-nerve needle nerve conduction showed abnormality in the affected interdigital nerves in all definite IDN cases and confirmed the diagnosis of IDN in 10 cases by the abnormal dip phenomenon (a selective decrease of 50% or more in the sensory CNAP amplitude of the affected nerve compared with that of the preceding interdigital nerve). In 11 possible IDN cases, IDN was identified by the abnormal dip phenomenon. CONCLUSION: The near-nerve needle sensory nerve conduction of the interdigital nerves is a highly sensitive diagnostic test, and abnormal dip phenomenon is the most characteristic electrophysiological marker for the diagnosis of IDN. more...

The authors performed nerve conduction studies in nine PARK2 and eight idiopathic Parkinson disease patients and found a significant reduction of sural sensory nerve action potential (SNAP) amplitude in eight PARK2 patients who mostly remained asymptomatic. These data suggest that sensory axonal neuropathy may be a common clinical feature of PARK2 and a reduced amplitude of sural SNAP could be a diagnostic indicator of PARK2. more...

Various parameters of the repetitive nerve stimulation (RNS) test of the abductor digiti quinti muscle were analyzed statistically in 34 patients with Lambert-Eaton myasthenic syndrome (LEMS). The sensitivity and specificity of the increments after exercise and after 50-HZ stimulation for the diagnosis of LEMS were compared with reference values in 40 normal subjects and data from 538 tests in patients with myasthenia gravis (MG). When we used a 100% increment (the "gold standard") as the normal limit for the postexercise facilitation (PEF) or the high-rate stimulation (HRS) test, the diagnosis of LEMS was confirmed in 29 (85%) cases. When a 60% increment was used as the normal limit, the diagnosis of LEMS was made in 97% of cases. In MG, a 60% increment was observed in only 4 of 538 cases by HRS and in none by the exercise test. Thus, the use of a 60% increment showed a sensitivity of 97% for the diagnosis of LEMS and a specificity of 99% in excluding MG. A 60% increment in either the PEF or HRS test for the diagnosis of LEMS is a desirable alternative to the 100% increment previously considered to be the gold standard for this diagnosis. more...

Objective: To report the clinical, electrophysiologic, and histologic characteristics of subacute inflammatory demyelinating polyneuropathy (SIDP) and to present the diagnostic criteria of this disease. Methods: For a diagnosis of “definite SIDP,” there were four mandatory criteria: 1) progressive motor and/or sensory dysfunction consistent with neuropathy in more than one limb with time to nadir between 4 and 8 weeks, 2) electrophysiologic evidence of demyelination in at least two nerves, 3) no other etiology of neuropathy, and 4) no relapse on adequate follow-up. Supportive criteria included high spinal fluid protein level (>55 mg/dL) and inflammatory cells in the nerve biopsy. A diagnosis of “probable SIDP” required progression of demyelinating neuropathy over a 4- to 8-week period. Results: Sixteen definite SIDP patients were identified among 29 probable SIDP patients. An antecedent infection was found in 38% of cases. The two most common neuropathy types were a symmetric motor–sensory neuropathy and a pure motor neuropathy. Cranial nerve deficits and respiratory failure were rare. Spinal fluid protein was high in 93% of cases. Demyelination was documented by the motor nerve conduction in 88% of cases and by the near-nerve needle sensory nerve conduction in two cases. Almost all patients were treated with prednisone and some with additional immunotherapies. Complete recovery was achieved in 69% of cases and partial recovery in others. Definite SIDP had all the characteristics of CIDP with three exceptions: a higher rate of antecedent infection, no relapse rate, and a high rate of recovery to normal. Conclusion: Subacute inflammatory demyelinating polyneuropathy is a definite entity bridging the gap between Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathy. more...

Motor involvement by varicella zoster virus (VZV) in the extremities is relatively rare and may be due to the extension of inflammation to the anterior horn or anterior motor roots.1 In this study we observed conduction block of the peripheral nerves and lesions in the right upper extremity on MRI in a patient with VZV infection. A 64-year-old woman noticed swelling of her right index finger followed by dysesthesia, reddening of the skin, and vesicle in the area from the right shoulder to the palm. She was diagnosed with herpes zoster and treated with acyclovir IV. She had been treated for chronic myelocytic leukemia (CML, chronic stage) since 1992. Four weeks later she noticed weakness of her right upper extremity that progressed slowly. She was admitted to our hospital 2 months later. Physical examination revealed pigmentation of skin in the right C6 and C7 dermatomes. Cranial nerves were intact. Manual muscle testing revealed weakness in the right upper extremity, which did not correspond to individual myotomes: deltoid (4/5), biceps (4/5), brachioradialis (4/5), triceps (3/5), extensor … more...

Background: We investigated the effect of age on nerve conduction parameters with special reference to the compound muscle action potential (CMAP) duration and duration ratio. Method: We examined 295 subjects (aged 15–85 years old) with no previous history or present signs of peripheral neuropathy. The subjects were divided into 3 groups: young (15–34 years old); intermediate (35–64 years old), and old (65–85 years old). Results: CMAP amplitude was lower in the old group than in the young group; however, the CMAP ratio (proximal CMAP/distal CMAP) did not change with age. The CMAP duration and duration ratio did not differ among the 3 groups. The CMAP area was smaller in the old group, but the area ratio was almost constant among the 3 groups. Conclusion: We suggest that age-related changes in CMAP amplitude, duration or area may occur uniformly, at least in the forearm and the calf segment, in routine nerve conduction studies. The present findings also provide useful and reliable information, regardless of age, in diagnosing peripheral neuropathy. more...

QTc intervals were measured using an electrocardiogram and other autonomic function tests, in 66 neuropathy patients with non-insulin-dependent diabetes mellitus (59.0±12.5 years; mean ± SD). The change in R-R interval did not influence the QTc interval, as calculated by the equation: QTc =QT+(1000-R-R)/7 (ms), compared with the conventional Bazett's equation which appeared to overcompensate in the case of a small R-R interval. The QTc interval in the diabetic patients was significantly longer than that in age-matched controls. The QTc interval showed an inverse correlation with the coefficient of variation of the R-R interval and skin blood flow at rest. However, no correlation was found between QTc interval and blood pressure change, change in heart rate on standing, or results of the sympathetic skin response. The QTc interval did not correlate significantly with motor or sensory nerve conduction parameters. We conclude that the QTc interval can be a simple and useful autonomic indicator for diabetic neuropathy relatively independent of other abnormalities of autonomic and somatic nervous system function. Clin Auton Res 8:139–143 more...

Unilateral enlargement of the calf in a 57-year-old man with S1 radiculopathy is described in this case report. Short tau inversion recovery-weighted imaging provided evidence of selective hypertrophy and a high signal intensity of the gastrocnemius and soleus. Histopathological analysis of the gastrocnemius revealed an endomysial inflammatory infiltrate and marked denervation lesions. Marked signs of denervation are suggestive of focal myositis secondary to neurogenic damage. The patient was treated with an oral corticosteroid (30 mg/day) and the calf hypertrophy was dramatically reduced within 5 weeks. Our case indicates that steroid therapy should be tried because it may be a potentially treatable disease. more...

A 91-year-old woman felt tingling sensation in her foot three weeks ago, followed by tingling in the hands. Soon after, her gait progressively had become unsteady. Neurological examination revealed weakness of bilateral hand muscles and toe extensors (3/5 in MRC scale), muscle wasting in hands, areflexia, superficial sensory loss showing glove-and-stocking type distribution, decreased vibration and proprioceptive sensation, and severe sensory ataxia. Cerebrospinal fluid showed 1.3 WBC/μl and 50 mg/dl protein. Guillain–Barre syndrome was suspected, although muscle wasting in her hands was atypical. Nerve conduction studies (NCS) demonstrated chronic motor and sensory demyelinating neuropathy with disproportionate distal slowing. The patterns of nerve conduction abnormalities were similar to atypical CIDP (distal acquired demyelinating symmetric neuropathy). She was treated by intravenous immunoglobulin (IVIg), then her gait improved soon after. A repeated NCS for four months after treatment showed the decrease of the distal latencies. However, nerve conduction velocity was still around 20 m/s for all examined nerves, suggesting a uniform demyelination. Anti-ganglioside and myelin associated glycoprotein antibodies were negative. Gene analysis demonstrated the DNA duplication in chromosome 17p11.2–p12. She was finally diagnosed as CMT1A associated with acquired demyelinating neuropathy (ataxic Guillain–Barre syndrome). more...