Clinical Neurology最新文献

We report a patient with posterior cortical atrophy (PCA) who manifested as agraphia for kanji and statokinetic dissociation (Riddoch phenomenon). A 52-year-old, right-handed woman complained that beginning at age 50, she could write only kana (a phonographic script) but not kanji (a morphographic script). She could not write even her own name in kanji. Neuropsychologic examinations disclosed kanji-dominant agraphia, acalculia, right-left disorientation, finger agnosia, constructional apraxia, and simultanagnosia. Many of these, including agraphia, are components of Gerstmann syndrome. She manifested no aphasia or alexia, while not only writing but also copying of kanji was impaired. Speech functions, behavior, and personality were relatively spared. The patient also displayed statokinetic dissociation (Riddoch phenomenon): kinetic Goldmann fields were normal, but static Humphrey visual fields showed an incongruous right homonymous hemianopsia. MRI showed atrophy of the left parietal lobe. ^99mTc ethyl cysteinate dimer (ECD) single-photon emission computed tomography (SPECT) showed hypoperfusion , predominantly in the left hemisphere and especially left the parietal lobe . These clinical and neuroradiologic findings are consistent with PCA. In patients with PCA, suspected incomplete homonymous hemianopsia should be confirmed with a Humphrey visual field test. Ishihara pseudoisochromatic plates may not be reliable; color vision should be checked using the panel D-15 test.

A 17-years-old woman visited the hospital due to convulsions. T₂-weighted images showed high intensity areas in right temporal lobe and left frontal lobe. Enhanced T₁-weighted images showed mass-like lesions on the dura mater. Based on mononuclear pleocytosis and a reactive fluorescent treponemal antibody-absorption test in the cerebrospinal fluid, neurosyphilis and intracranial gumma were diagnosed, and antibiotic therapy was initiated. After treatments, the high intensity areas improved, and she had no recurrence of symptoms or MRI images. Intracranial gumma usually develops in tertiary syphilis, more than 1 year after infection. In this case, intracranial gumma developed within 1 year after infection. Even if the patient is a young woman, it is necessary to consider the possibility of intracranial gumma and select appropriate examinations and treatments earlier.

Encephalitis is a life-threatening disease with many causes. The continual discovery of newly identified forms of autoimmune encephalitis (AE) associated with antibodies to cell-surface or synaptic proteins has changed the paradigms for diagnosing and treating disorders. AE is one of the most common causes of non-infectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. These disorders can occur in patients with or without cancer. I review here the update of clinical management in AE. Recent clinical trends in AE include 1) the spread of clinical manifestations, 2) pitfalls of misdiagnosed cases and risk factors for misdiagnosis, and 3) treatment trends for refractory cases and symptomatic epilepsy. 1) The spread of clinical manifestations includes the presence of autoimmune psychosis (Pollak TA Lancet Psychiatry 2020), the presence of AE in adult-onset temporal lobe epilepsy (Kuehn JC, PLoS One 2020), and AE cases presenting with progressive dementia (Bastiaansen AEM, Neurol Neuroimmunol Neuroinflamm 2021). 2) Misdiagnosis and inappropriate use of diagnostic criteria for antibody-negative cases have been pointed out (Dalmau J. Lancet Neurol 2023). Misdiagnoses of AE occur for three reasons. First, non-adherence to reported clinical requirements for diagnostic criteria for AE. Second, the evaluation of inflammatory changes in head MRI and cerebrospinal fluid is insufficient. Third, absent or limited use of brain tissue assays along with use of cell-based assays that include only a narrow range of antigens. Red flags suggesting alternative diagnoses included an insidious onset, positive nonspecific serum antibody, and failure to fulfill AE diagnostic criteria. 3) Treatment trends for rituximab-resistant refractory cases include tocilizumab (IL6 receptor monoclonal antibody) and bortezomib (26S proteasome inhibitor). On the other hand, new Na channel inhibitors (lacosamide, etc.) and perampanel may be useful for treating symptomatic epilepsy in AE.

The patient is a woman in her 70s. Low back pain and left lower limb pain gradually worsened since 1 month ago, urinary retention and bilateral lower limb paralysis appeared and she was admitted to our department. Muscle weakness in both lower limbs, hypoesthesia and pain in both lower limbs predominantly in the right side, and loss of tendon reflexes in both lower limbs were observed. MRI showed severe lumbar deformity as well as swelling of the spinal conus medullaris, ring-shaped contrast effect, and contrast effect of the cauda equina nerve. Diffusion-weighted images of the spinal conus showed multifocal high signal. Cerebrospinal fluid showed 271 cells/mm³ (72% polymorphonuclear cells), 356 ‍mg/dl protein, 15 ‍mg/dl sugar, and negative bacterial culture. Suspecting bacterial intramedullary spinal abscess and cauda equina neuritis, she was started on intravenous Ceftriaxone (CTRX)/ Vancomycin (VCM) and oral MNZ. Thereafter, muscle weakness and sensory disturbance in both lower limbs gradually improved, and the patient was able to walk with a cane one month later. Cerebrospinal fluid and MRI findings gradually normalized. The diagnosis of bacterial intramedullary spinal cord abscess and cauda equina neuritis was made, which improved with conservative treatment.

A 57-year-old man presented with headache and fever, and was diagnosed as having aseptic meningitis on the basis of CSF pleocytosis. One month later, the symptoms became exacerbated, and lethargy also developed. Although general blood tests including electrolytes and creatine kinase showed no abnormalities, brain MRI with Gd-enhancement revealed enlargement of the whole pituitary gland, spreading to the stalk. Hormonal tests revealed pan-hypopituitarism. After ruling out diseases such as sarcoidosis, syphilis, tuberculosis, Sjögren syndrome and systemic lupus erythematosus, which could potentially cause hypophysitis, lymphocytic hypophysitis was diagnosed. Hormone replacement therapy ameliorated both the symptoms and the enlargement of the pituitary gland. This case was considered to be atypical lymphocytic hypophysitis, lacking abnormalities in general blood tests, which is essential when considering a differential diagnosis of aseptic meningitis.

We present a case of a 76-year-old woman diagnosed with pathologically confirmed progressive supranuclear palsy (PSP) with pallido-nigral-luysial atrophy, who initially presented with a dropped head. Upon her first visit, neurophysiological and neuroradiological examinations provided no definitive cause, and the tactile trick was effective, leading to a diagnosis of cervical dystonia. Trihexyphenidyl treatment had no effect, but her condition gradually improved over 3 years. By age 74, she developed gait freezing without muscle rigidity or tremor. Dopamine-transporter scintigraphy revealed reduced tracer uptake in the bilateral corpus striata, prompting the diagnosis of pure akinesia with gait freezing (PAGF). At age 76, the patient developed retrocollis, muscle rigidity in all extremities, and recurrent temporomandibular dislocation. She eventually died from aspiration pneumonia after several years of illness. At autopsy, the brain weighed 1,370 ‍g. Macroscopic examination showed atrophy of the pallidum and subthalamic nucleus and depigmentation of the substantia nigra. Histopathological analysis revealed degeneration with 4-repeat tau pathology in the substantia nigra, globus pallidus, and subthalamic nucleus, along with tufted astrocytes in the globus pallidus and putamen, confirming a pathological diagnosis of pallido-nigral-luysial atrophy-type PSP. We suggest that the clinical presentation of PAGF correlates well with the pathological findings of pallido-nigral-luysial atrophy. While dystonia in PSP is typically observed in the limbs, blepharospasm, or retrocollis, only two other cases of PSP with a dropped head have been reported. The pathophysiological mechanism remains unclear, but we hypothesize that 4-repeat tau pathology in the globus pallidus may contribute to the development of cervical dystonia. Further neuropathological studies are needed to confirm this hypothesis.

A 78-year-old male presented with abnormal behavior, which progressed to tonic-clonic seizures in right upper limb and impaired consciousness two weeks later. Initial brain MRI and cerebrospinal fluid findings were normal. However, on the 5th day, diffusion-weighted imaging revealed hyperintense areas in the left basal frontal lobe, striatum, and insular cortex. By the 12th day, T₁-weighted imaging demonstrated hyperintensity in the left striatum. The symptoms almost improved before the initiation of immunotherapy. Based on the time-course changes in MRI findings and positive serum leucine-rich glioma-inactivated 1 (LGI1) antibody results, the patient was diagnosed with anti-LGI1 encephalitis. The patient also had basal cell carcinoma. T₁ hyperintensity in the basal ganglia is a useful diagnostic feature of anti-LGI1 encephalitis.

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Syphilitic gumma is a rare manifestation of neurosyphilis that can cause mass lesions in the central nervous system. We present an atypical case of a 53-year-old man presenting with syphilitic gummas affecting both the brain and spinal cord. The patient presented with right facial numbness, worsening back pain, gait disturbances, and lower-limb weakness. Serological tests were positive for syphilis, and cerebrospinal fluid analysis showed elevated cell count, protein concentration, and positive syphilis tests. Brain and spinal cord MRI revealed dural-based enhancing mass lesions in the right middle cerebellar peduncle and conus medullaris. The patient underwent posterior decompression and biopsy of the conus medullaris. Histopathological findings excluded malignancy and were consistent with syphilitic gumma. The patient received intravenous benzylpenicillin, followed by oral amoxicillin, resulting in partial improvement of neurological symptoms and gradual regression of the lesions on follow-up MRI. This case highlights the importance of considering syphilitic gumma in the differential diagnosis of intracranial and spinal cord lesions in patients with syphilis. Prompt antibiotic treatment and serial MRI imaging are crucial for managing this condition.

Objective: Although population-based studies of hemiplegic migraine (HM) exist, large-scale clinic-based studies focusing on the detailed clinical characteristics of HM have not been reported. This study aims to define the clinical characteristics of HM in a tertiary care headache centre.

Methods: A retrospective analysis was conducted based on the medical records of HM patients.

Patients: This study included 163 consecutive HM patients who visited the National Hospital for Neurology and Neurosurgery between 2006 and 2013.

Results: According to the diagnostic criteria of International Classification of Headache Disorders (ICHD-3β), 142 patients were diagnosed with HM. Although 21 patients did not satisfy the diagnostic criteria, migrainous headaches with repetitive hemiparesis were reported and other disorders were excluded, hence these patients were clinically diagnosed with HM. The temporal progression of aura symptoms was atypical in 40 patients. The median duration of hemiparesis was 24 hours (interquartile range: 3-60 hours) which was far longer than that of previous population-based studies. The lifetime experience of an episode of motor aura exceeding 72 hours was reported in 51.6%. Hemiparesis was observed without full recovery in 9 patients (5.5%).

Conclusions: In many HM patients, the temporal progression of aura symptoms was diverse compared to typical descriptions in the literature, and the aura symptoms sustained longer than reported in the population-based study.