A questionnaire survey was conducted on 8,402 members of the Japanese Neurological Society to examine the current status and countermeasures for physician burnout, and 1,261 respondents (15.0%) responded. In this paper, we report the results of a comparison between male and female physicians. There was a significant difference in working and living conditions only for married people. It was confirmed that men work under stricter conditions in terms of working hours, and that the burden on women is heavier in the division of housework. Analysis using the Japanese Burnout Scale revealed no gender differences in overall scores, but as for factors related to burnout, in addition to factors common to both men and women, factors specific to men or women were clarified.
A 62-year-old Japanese man with swollen fingers and walking difficulty due to myalgia and muscle weakness in proximal limb muscles was admitted to our hospital. Serum creatine kinase was remarkably increased (7,380 U/l) and rapidly progressing interstitial pneumonia developed. Muscle biopsy showed necrotic and regenerating fibers without mononuclear infiltration and fibrosis. Anti-Th/To antibodies were detected in the serum, and anti-Th/To antibody-positive systemic sclerosis was diagnosed. Anti-Th/To antibody-positive sclerosis-associated myopathy has not yet been reported in the literature. The present case suggests that anti-Th/To antibody-positive systemic sclerosis can be accompanied by immune-mediated necrotizing myopathy and be effectively treated with immunotherapy comprising corticosteroids, tacrolimus and immunoglobulin.
An 80-year-old woman presented with sudden-onset dysarthria and left-side dominant quadriparesis and transferred to our hospital. A neurologic examination revealed slurred speech, prominent left upper extremity weakness and mild weakness of the other extremities. Brain MRI revealed a history of right-side cerebral artery bypass surgery, but no new lesions indicative of stroke. Left upper extremity weakness had improved soon after admission, so a transient ischemic attack was suspected. After admission, the dysarthria fluctuated. The patient’s respiratory condition deteriorated several days later and she required ventilation support. Assessment of the cause of the respiratory failure revealed positive muscle-specific kinase (MuSK) antibodies, which suggested myasthenia gravis (MG). The symptoms gradually improved with immunotherapy and we were able to completely withdraw her from the ventilator after a few months. There were some reports that dysphagia and dysarthria present suddenly like stroke without fluctuation of symptoms in elderly-onset MG. It is necessary to note that MG diagnosis may be difficult if elderly patients have multiple comorbidities and unclear diurnal fluctuations.
A 78-year-old woman was diagnosed with herpes zoster in the first branch of the trigeminal nerve and was treated with amenamevir. Subsequently, she was hospitalized for postherpetic neuralgia. Fever and unconsciousness were observed, and a diagnosis of varicella-zoster virus meningoencephalitis and vasculitis was made. In addition to the antithrombotic therapy, she was treated with intravenous acyclovir and steroid pulse therapy; however, her unconsciousness persisted. Amenamevir was not transferrable to the spinal fluid and resulted in an incomplete treatment of herpes zoster in the cerebral nerve region, suggesting that this case may be related to the severe course of the disease.
A 71-year-old woman was admitted to our hospital with type2 respiratory failure. Her daily life activities had been normal, although she had noticed mild truncal weakness in her sixties. Her parents were consanguineous, and her sister had suffered similar symptoms. Although Pompe disease was suspected on the basis of the clinical course and CT findings of selective muscular atrophy in the paraspinal, thigh flexor and sartorius muscle, acid alpha-glucosidase activity was normal. The serum creatine kinase level was not elevated, and muscle biopsy showed no specific change. Genetic analysis revealed a novel homozygous variant c.227T>C (p.Phe76Ser) in the SELENON gene, and she was suspected to have selenoprotein-related myopathy, which is reported to develop in childhood. Selenoprotein-related myopathy should be considered as a differential diagnosis in aged patients presenting with respiratory failure of unknown origin.
We experienced 3 adult patients with intractable focal epilepsy treated by levetiracetam (LEV) as polytherapy, who showed paradoxical effect (PE). Starting dose of LEV was small (62.5, 250 mg/day) and we gradually increased by less than 250 mg/day, every more than 2 weeks. Within 6 months after LEV was added, LEV of 750 to 1,000 mg/day brought reduction of seizure frequency. Serum concentration of LEV was 13.3 and 14.0 μg/ml. In order to obtain better seizure control, LEV was increased up to 1,000–2,500 mg/day (19.3–35.0 μg/ml) within one year, and they developed PE. They all showed increased habitual seizures, occurring in cluster. Once dose of LEV deceased down to what produced the maximum seizure suppression, all of the patients regained the better seizure control. It is most likely that at least in some patients like present 3 cases, PE of LEV may express U curve association between dose and effect and that it was only delineated by slow titration.
A 35-year-old Sudanese man experienced bitter tastes on the right side of his tongue from January 2012. He was admitted to our hospital in March 2012 because of the appearance of distress, right facial palsy, nausea, and dizziness from late February 2012. A neurological examination revealed Bruns nystagmus, which increased on rightward gaze, as well as total hypoesthesia in the distribution of the maxillary branch of the right trigeminal nerve, moderate right peripheral type facial nerve palsy, and limb ataxia on the right side. Neither muscle weakness nor sensory disturbance was observed. Slight hyperreflexia was noted in the right extremities, and bilateral plantar responses were flexor. He showed wide-based ataxic gait and was unable to do tandem gait. Brain CT scans and magnetic resonance (MR) images revealed a mass lesion in the right pons to the right middle cerebellar peduncle with ring enhancement, suggestive of a “target” sign. Laboratory tests, including hematological and biochemical analyses, tumor markers, and antibodies, had normal values while the tuberculin reaction and QuantiFERON-TB Gold were strongly positive. Cerebrospinal fluid analysis revealed a slight increase in the protein level (76 mg/dl) with a normal cell count (2 per μl), and polymerase chain reaction-based tests and cultures were negative for Mycobacterium tuberculosis three times. Right subclavicular lymph node and right adrenal gland showed accumulation of fluorodeoxyglucose on positron emission tomography-CT, as did the mass lesion in the brainstem. These findings suggested a possibility of a metastatic malignant tumor or extrapleural tuberculoma. Because of the patient’s religious belief, we were unable to perform a biopsy of the lymph node, and thus administered anti-tuberculous drugs. With treatment, his neurological symptoms such as facial palsy and ataxia improved steadily except for paradoxical worsening for the initial five days, and the gadolinium-enhanced lesion shrunk markedly. Follow-up MR images demonstrated that the lesions did not expand further for 9 months. From this course of treatment, we diagnosed the patient’s tumor as brainstem tuberculoma. Brain tuberculoma sometimes resembles a malignant tumor, and it is therefore challenging to diagnose brainstem tuberculosis in cases without lung lesions. It is important to make a comprehensive diagnosis based on the patient’s background, imaging, and course of treatment, and to treat brainstem tuberculoma promptly.
We report a 47-year-old woman who presented with progressive myalgia, weakness in the proximal limbs, and dysphagia for a month and a half. No skin rash was observed on admission. Examination of MRI data suggested inflammatory changes in the proximal limbs and trunk muscles. Biopsy specimens from the left biceps muscle showed no perifascicular atrophy, but immunohistochemical staining revealed the presence of myxovirus resistance protein A (MxA) in myofibers, strongly suggesting dermatomyositis (DM). In addition, her serum was positive for anti-nuclear matrix protein 2 (anti-NXP-2) antibody, which is reportedly useful as a marker of DM without skin lesions. Her symptoms gradually improved upon intravenous methylprednisolone pulse therapy in conjunction with oral prednisolone, oral tacrolimus, and intravenous immunoglobulin therapy. Our findings suggest that in cases where inflammatory muscle disease is suspected, anti-NXP-2 antibody analyses should be considered for precise diagnosis, even if there are no dermatological symptoms.
A 33-year-old man with an unremarkable family history has had limb muscle weakness, joint contracture and skeleton deformation from early childhood. He was diagnosed with spinal muscular atrophy (SMA) by a pediatrician. He needed assistance and used orthoses in his daily life. There was no subjective sensory disturbance. However, physical examination showed slight sensory impairment, and nerve conduction study indicated sensory motor axonal neuropathy. This finding suggested Charcot-Marie-Tooth disease (CMT). Gene analysis detected MORC2 S87L mutation, leading to a diagnosis of CMT type 2Z. Patients with MORC2 S87L mutation are known to exhibit a severe phenotype, and may mimic SMA. It is important to demonstrate subclinical sensory neuropathy in patients with MORC2 S87L mutation mimicking SMA.