Battey's operation as a treatment for hysteria: a review of a series of cases in the nineteenth century.

Ovarian resection as a treatment for hysteria, called 'Battey's operation' or 'normal ovariotomy', was performed in the nineteenth century. Battey later reported that the resected ovaries appeared to have 'cystic degeneration'. Currently, patients with acute neuropsychiatric symptoms are screened for teratomas for the differential diagnosis of anti-NMDA receptor encephalitis. There is now a hypothesis that ovarian lesions resulting in paraneoplastic encephalitis were among the patients who underwent Battey's operation. We identified 94 published cases of Battey's operation for neuropsychiatric symptoms in the late nineteenth century. Among 36 cases with detailed descriptions, we found 3 patients who showed acute onset neuropsychiatric symptoms with macropathological ovarian findings that were compatible with teratoma. They showed favourable prognoses after surgery and might have motivated the surgeons to perform the operation.

Clinical and neurophysiological variability in Andersen-Tawil syndrome.

INTRODUCTION: Andersen-Tawil syndrome (ATS) is characterized by a triad of periodic paralysis, ventricular arrhythmias, and dysmorphism. However, patients often lack one or more of these features. METHODS: Clinical and neurophysiological features were reviewed of five members in two families with heterozygous mutations in KCNJ2 (R218Q and R67W). RESULTS: Only one patient had all features of the triad of ATS. One patient had low-set ears, and the others had minor anomalies. Bidirectional ventricular tachycardias were seen in two patients. Two patients (R67W) never had episodes of paralysis. The long exercise test was abnormal in three patients with episodes of paralysis, but normal in two without paralytic episodes. DISCUSSION: ATS patients without skeletal muscle symptoms can have normal neurophysiological examinations. They can show variability in phenotype or the severity of arrhythmias. Such variability among patients who share the same gene mutations may result in underdiagnosis of ATS.

Isolated body lateropulsion in a patient with pontine infarction.

A 72-year-old man with diabetes mellitus and hypertension was admitted to our hospital with lightheadedness. The patient showed lateropulsion to the right side, but his neurological findings were otherwise normal. Brain magnetic resonance images showed a fresh ischemic infarct in the left dorsal part of the lower pons. Body lateropulsion is characterized by an irresistible falling to one side and has been reported in lesions in several brain regions. However, it has rarely been reported in pontine lesions. We suggest that physicians should be aware that pontine lesions can cause isolated body lateropulsion without other neurological deficits.

Recurrent aseptic meningitis in association with Kikuchi-Fujimoto disease: case report and literature review.

BACKGROUND: Kikuchi Fujimoto disease (KFD), or histiocytic necrotising lymphadenitis, is a benign and self-limiting condition characterised by primarily affecting the cervical lymph nodes. Recurrent aseptic meningitis in association with KFD is extremely rare and remains a diagnostic challenge. CASE PRESENTATION: We report a 28-year-old man who presented 7 episodes of aseptic meningitis associated with KFD over the course of 7 years. Histopathological findings of enlarged lymph nodes led to the diagnosis of KFD. The patient's headache and lymphadenopathy spontaneously resolved without any sequelae. CONCLUSIONS: A diagnosis of KFD should be considered when enlarged cervical lymph nodes are observed in patients with recurrent aseptic meningitis. A long-term prognosis remains uncertain, and careful follow-up is preferred.

Sleep-related hallucinations in patients with Parkinson's disease.

Given that sleep-wake cycle dysfunction can cause hallucinations in Parkinson's disease patients, sleep-related hallucinations may be a different subtype from hallucinations that occur only during full wakefulness. However, few studies that distinguish the onset situations of hallucinations related to sleep from those that occur in full wakefulness have been conducted to investigate hallucinations in Parkinson's disease patients. Therefore, we conducted a multicenter observational study to investigate the prevalence of and factors associated with sleep-related hallucinations in patients with Parkinson's disease. Information on hallucinations was collected by using a questionnaire and face-to-face interviews. Of 100 consecutive patients with Parkinson's disease, 29 (29%) reported sleep-related hallucinations, and 16 (16%) reported hallucinations only in the full wakefulness. A longer duration of Parkinson's disease treatment (OR 1.35, 95% CI 1.07 to 1.72), higher Beck Depression Inventory-II scores (OR 1.07; 95% CI 1.01 to 1.14), and higher rapid eye movement sleep behavior disorder scores (OR 5.60; 95% CI 1.54 to 20.38) were independent factors associated with the presence of sleep-related hallucinations in a multivariable analysis. Sleep-related hallucinations, but not daytime hallucinations, were associated with probable rapid eye movement sleep behavior disorder. Phenomenological discrimination between sleep-related hallucinations and daytime hallucinations is important for elucidating the full pathology in Parkinson's disease and the mechanisms underlying hallucinations.

Hystero-epilepsy in the Tuesday Lessons and NMDA receptor function: A hypothesis for dissociative disorder.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis patients demonstrate characteristic multistage progression and movement disorders, which are analogous to hystero-epilepsy in Jean-Martin Charcot's Tuesday Lessons. First, based on a review of the Tuesday Lessons recorded by Charcot's pupils, we hypothesized that there were patients with anti-NMDAR encephalitis among those diagnosed with hystero-epilepsy in the nineteenth century. We found acute-onset multiple neuropsychiatric manifestations resembling anti-NMDAR encephalitis among patients with hystero-epilepsy. Patients with drug withdrawal syndrome, dissociative and conversion disorders and patients under hypnosis from the modern point of view were also identified. These results suggested that hystero-epilepsy in the Tuesday Lessons could encompass dissociative and conversion disorders, hypnosis, drug withdrawal syndrome, and anti-NMDAR encephalitis-like manifestations. Based on Charcot's observations and current progress in molecular biology, such as the identification of glutamate/NMDAR system dysfunction in drug withdrawal syndrome, we then hypothesized that patients with dissociative and conversion disorders and those under hypnosis could also have hypofunction of the glutamatergic system. The NMDAR hypofunction hypothesis is emerging as a pathogenesis of schizophrenia. NMDAR antagonists are known to evoke symptoms similar to schizophrenia, anti-NMDAR encephalitis and near-death experiences. In current clinical reports, spectrum disorders such as dissociative disorder and conversion disorder have been observed in patients with anti-NMDAR encephalitis. Our hypothesis will offer an expansion of the NMDAR hypofunction hypothesis from psychosis to functional neurological disorders and normal specific situations, such as hypnosis, thanatosis, and near-death experiences.

Impact of the COVID-19 Pandemic on the Quality of Life of Patients with Parkinson's Disease and Their Caregivers: A Single-Center Survey in Tochigi Prefecture.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has negatively affected the mental health of the general population. OBJECTIVE: We investigated the determinants of quality of life (QOL) in Parkinson's disease (PD) patients during the COVID-19 pandemic. METHODS: Impacts of lifestyle changes due to the COVID-19 pandemic on 100 patients with PD and their caregivers/spouses were assessed. The Hospital Anxiety and Depression Scale was used to assess anxiety and depression. The physical component summary (PCS) and mental component summary (MCS) scores of the short form (SF)-8 were used to evaluate health-related QOL. RESULTS: Regarding health-related QOL, physical function, role physical, general health, vitality and the PCS score were significantly worse in PD patients than in caregivers. Worsening of PD-related symptoms, increased stress, and decreased physical activity were observed in 29.0%, 37.0% and 44.0% of PD patients, respectively. Sixteen patients (16.0%) experienced problems with hospital access, but none reported medication shortages. Strong concerns about COVID-19 were reported by 47.0% of caregivers and 50.0% of PD patients. In PD patients, increased gait disturbance and rigidity, disease severity, smoking, the levodopa equivalent dose and decreased body weight predicted a worse PCS score; anxiety, depression, female sex, stress and long disease duration predicted a worse MCS score. In caregivers, age and smoking contributed to a worse PCS score; depression, stress and worsening patient mood contributed to a worse MCS score. CONCLUSION: We report the negative impacts of the COVID-19 pandemic on health-related QOL and its determinants in PD patients and their caregivers.

A family with adult-onset myofibrillar myopathy with BAG3 mutation (P470S) presenting with axonal polyneuropathy.

We report a family with adult-onset myofibrillar myopathy with BAG3 mutation who presented peroneal weakness and axonal polyneuropathy, mimicking axonal Charcot-Marie-Tooth disease. The male proband noticed difficulty in tiptoeing at age 34. At age 42, the examination showed muscle weakness and atrophy in distal lower extremities with diminished patellar and Achilles tendon reflexes. Thermal and vibration sensations were also impaired in both feet. The serum CK level was 659 U/L. On muscle imaging, predominant semitendinosus muscle atrophy coexisted with atrophies in the quadriceps, gastrocnemius and lumbar paraspinal muscles. The muscle biopsy showed myofibrillar myopathy with fiber type grouping. His 68-year-old mother also had suffered from distal leg weakness and sensory impairment since her forties. A heterozygous mutation in BAG3 (P470S) was identified in both patients. Clinical features of myofibrillar myopathy with axonal polyneuropathy were consistent with BAG3-related myopathy. Our patients showed remarkably mild presentations without cardiomyopathy, unlike the majorities of previously reported cases.

[Selective muscular atrophy in a family with hereditary myopathy with early respiratory failure].

Hereditary myopathy with early respiratory failure (HMERF) with heterozygous mutations in the titin gene (TTN) is characterized by respiratory failure developing from the early phase of limb weakness or gait disturbance. Here, we describe a characteristic distribution of muscle involvement in three members of a HMERF family with a TTN mutation. Despite the differences in severity exhibited among the father, daughter and son, the systemic imaging studies showed a similar pattern among these individuals. The semitendinosus and fibularis longus muscles were selectively affected, as described previously. In addition, we found marked atrophy in the sternocleidomastoid and psoas major muscles, regardless of the disease severity. The atrophy in selective trunk muscles observed in routine CT scans can be useful for the differential diagnosis of hereditary myopathies with heart and respiratory failure.

[Case of atopic myelitis with acute onset and atypical distribution of spinal cord lesions].

A 23-year-old woman was admitted to our department because of gait disturbance, sensory impairment in the lower limbs, and sphincter disturbance, all of which had been developing within 24 hours before admission. Neurological examination disclosed symmetric muscle weakness, sensory impairment, and diminished tendon reflexes in the lower limbs. The urinary bladder was hypoactive, and the anal tone was reduced. The spinal cord MRI performed on the day of admission revealed swelling of the epiconus. The CSF findings were not remarkable, except for the elevated levels of IgE (8 IU/ml) and MBP (7.8 ng/ml). Besides, there was a marked increase in the serum mite-specific IgE titers. Collectively, we made a diagnosis of atopic myelitis. She was treated with steroid pulse therapy and plasma exchange, which led to a significant amelioration of her neurological manifestations. The repeat MRI carried out on the 21st day of her admission displayed several foci scattered in the lumbar and sacral spinal cord segments, which exhibited high intensity signals on the T2-weighted images. The values of IgE and albumin in the CSF and serum raised the possibility of intrathecal IgE synthesis. We measured her CSF IgE levels at several time points during admission. The temporal profile of her CSF IgE levels was not correlated with that of her neurological disabilities.

The crucial role of Campylobacter jejuni genes in anti-ganglioside antibody induction in Guillain-Barre syndrome.

Molecular mimicry of Campylobacter jejuni lipo-oligosaccharides (LOS) with gangliosides in nervous tissue is considered to induce cross-reactive antibodies that lead to Guillain-Barre syndrome (GBS), an acute polyneuropathy. To determine whether specific bacterial genes are crucial for the biosynthesis of ganglioside-like structures and the induction of anti-ganglioside antibodies, we characterized the C. jejuni LOS biosynthesis gene locus in GBS-associated and control strains. We demonstrated that specific types of the LOS biosynthesis gene locus are associated with GBS and with the expression of ganglioside-mimicking structures. Campylobacter knockout mutants of 2 potential GBS marker genes, both involved in LOS sialylation, expressed truncated LOS structures without sialic acid, showed reduced reactivity with GBS patient serum, and failed to induce an anti-ganglioside antibody response in mice. We demonstrate, for the first time, to our knowledge, that specific bacterial genes are crucial for the induction of anti-ganglioside antibodies.

Temporal changes in the documentation of neurological findings among patients with acute ischaemic stroke in a single centre in Japan: a retrospective cross-sectional study.

OBJECTIVE: To evaluate temporal differences in the documentation of neurological findings by the same physicians in patients with ischaemic stroke while in hospital. We also investigated differences in the rate of documentation of neurological findings in patients with stroke between internists and neurosurgeons. DESIGN: A retrospective medical chart review. PARTICIPANTS: Hospitalised adult patients with acute ischaemic stroke who stayed 7 or more days in our hospital. Neurosurgeons (n=8) and internists (n=19) caring for these patients (including up to 10 patients per physician). MAIN OUTCOME MEASURES: The documentation rate of any neurological finding in the patients on each day (from day 1 to 7 and on discharge). The documentation rates of eight neurological finding components (consciousness, mental status, cranial nerves, motor function, sensory function, coordination, reflexes and gait). We included only documentation by the same physician. Fisher's exact test was used to evaluate differences in outcomes between neurosurgeons and internists. RESULTS: During the study period, we identified 172 patients with stroke who were cared for by 27 physicians. The documentation rates of any neurological findings were 94% (day 1), 58% (day 2), 35% (day 3), 40% (day 4), 32% (day 5), 30% (day 6) and 23% (day 7). On discharge, all eight neurological finding components were documented in less than 10% of all cases. The documentation rate was significantly higher by internists than that by neurosurgeons on each day but not on discharge. CONCLUSIONS: The documentation rate of neurological findings by physicians during usual stroke care decreased to less than 50% after the third hospital day. Given the importance of temporal changes in the neurological symptoms of patients with stroke, further study is needed to determine whether this low documentation rate after the third hospital day was due to a lack of physician interest in neurological findings or other factors.

Demyelinating hypertrophic inferior alveolar nerve mimicking a nerve tumor.

We herein report a patient with demyelinating inferior alveolar nerve hypertrophy, which was initially suspected to have a nerve tumor. A 39-year-old woman with childhood-onset polyneuropathy presented with tooth pain and visited a dental clinic. An X-ray examination of the mandible revealed enlargement of the mandibular canal, and a nerve tumor was suspected. CT scan and MRI showed hypertrophy of the inferior alveolar nerve along its entire length. We diagnosed the patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which was supported by the spontaneous recovery reported in her childhood, the results from a nerve conduction study and MRI data. CIDP should be considered in the differential diagnosis of mandibular canal enlargement.

Acute hemicerebellitis in a young adult: a case report and literature review.

Acute hemicerebellitis, marked by headache with or without cerebellar signs, is a rare clinical entity involving a unilateral cerebellar hemisphere. The pathogenesis of acute hemicerebellitis remains unclear, and the disease rarely occurs in adults. Here, we report an 18-year-old woman who presented with a lack of coordination of the right hand and leg lasting longer than one week, following a pulsatile headache. A neurological examination disclosed ocular dysmetria, right-sided limb ataxia and slight truncal ataxia. Cerebrospinal fluid analysis showed mononuclear pleocytosis. The serology and autoimmune studies were unremarkable. Brain magnetic resonance imaging (MRI) revealed a focal signal change in the right cerebellar hemisphere and vermis. Acute hemicerebellitis was diagnosed, and the patient was treated with intravenous methylprednisolone sodium succinate and acyclovir. Subsequently, the headache resolved, and the cerebellar signs were markedly improved. Twenty days after admission, she became asymptomatic and brain MRI showed resolution of cerebellar hyperintensity on the right side. In conclusion, we identified only 6 additional patients with adult-onset acute hemicerebellitis from previous reports, highlighting the importance of recognizing this rare clinical entity. Its clinical outcome is usually favorable, but in the acute phase, attention should be directed toward clinical symptoms that are suggestive of increased intracranial pressure.

Selective muscle involvement in a family affected by a second LIM domain mutation of fhl1: an imaging study using computed tomography.

Mutations in the four-and-a-half LIM domains 1 gene (fhl1) are associated with various phenotypes of hereditary myopathies, including reducing body myopathy. We describe here a mother, daughter and son suffering from FHL1 myopathy with a mutation in the second LIM domain of fhl1. We investigated whether there is a characteristic muscle involvement in both sexes. Despite the variety of symptoms exhibited by the male and female patients, the systemic imaging studies showed a similar pattern: the flexor muscles of the brachium and thigh were affected earlier than the extensor muscle with a profound degeneration of the paraspinal muscles. These findings may include one of the characteristic clinical features for suspecting a mutation in the second LIM domain.

Hypothermia, hypotension, hypersomnia, and obesity associated with hypothalamic lesions in a patient positive for the anti-aquaporin 4 antibody: a case report and literature review.

OBJECTIVE: To describe a patient positive for the anti-aquaporin 4 antibody with hypothalamic lesions showing hypothermia, hypotension, hypersomnia, and obesity. DESIGN: Case report. SETTING: University hospital. PATIENT: We describe a 21-year-old woman who was positive for anti-aquaporin 4 antibody and presented with hypothermia, hypotension, and hypersomnia owing to bilateral hypothalamic lesions as the only abnormal clinical finding. RESULTS: Immediate steroid administration resulted in significant improvement of the patient's vital signs and imaging findings; however, her cognitive impairment and sleepiness persisted, and she subsequently developed obesity. Decreased cerebrospinal fluid orexin levels and sleep studies confirmed the diagnosis of narcolepsy due to medical condition. Physicians should be aware that neuromyelitis optica spectrum disorders can initially involve the hypothalamus. CONCLUSIONS: We emphasize that measurement of anti-aquaporin 4 antibody is of clinical importance in the differential diagnosis of hypothalamic lesions.

Ganglioside mimicry as a cause of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS), characterized by acute progressive limb weakness and areflexia, is the prototype of postinfectious autoimmune diseases. Campylobacter jejuni is the most frequently identified agent of infection in GBS patients, often preceding acute motor axonal neuropathy (AMAN), a variant of GBS. Anti-GM1, anti-GM1b, anti-GD1a, and anti-GalNAc-GD1a IgG antibodies are associated with AMAN. Carbohydrate mimicry [Galbeta1-3GalNAcbeta1-4(NeuAcalpha2-3)Galbeta1-] was seen between the lipo-oligosaccharide of C. jejuni isolated from an AMAN patient and human GM1 ganglioside. Sensitization with the lipo-oligosaccharide of C. jejuni induces AMAN in rabbits as does sensitization with GM1 ganglioside. Paralyzed rabbits have pathological changes in their peripheral nerves identical to changes seen in human GBS. C. jejuni infection may induce anti-ganglioside antibodies by molecular mimicry, eliciting AMAN. This is the first verification of the causative mechanism of molecular mimicry in an autoimmune disease. To express ganglioside mimics, C. jejuni requires specific gene combinations that function in sialic acid biosynthesis or transfer. The knockout mutants of these landmark genes of GBS show reduced reactivity with GBS patients' sera, and fail to induce an anti-ganglioside antibody response in mice. These genes are crucial for the induction of neuropathogenic cross-reactive antibodies. An approach for evaluating intravenous immune globulin, a treatment for GBS, based on our animal model of AMAN is also discussed in this review, and recent advances made in this field are described.