Brain and nerve = Shinkei kenkyū no shinpo
-
We report a 47-year-old woman who developed a thunderclap headache. Head axial, fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) revealed high signal lesions in the left occipital and right parietal lobes. Apparent diffusion coefficient mapping showed a vasogenic edema pattern. ⋯ Calcium channel blocker use was continued and vasoconstriction improved by day 70. In this case, the presenting symptom was thunderclap headache, which is a characteristic feature of reversible cerebral vasoconstriction syndrome (RCVS). Therefore, PRES may be caused by RCVS.
-
Continuous infusion of intrathecal baclofen (ITB), via implanted pump, is a powerful tool in the management of severe spasticity in neurological disorders. The advantages of ITB therapy are that it is non-destructive, reversible, and programmable, allowing for neuromodulation. ⋯ Few complications and adverse effects were reported. ITB therapy appears to be a promising treatment to improve residual motor control in patients with severe spasticity.
-
Review Case Reports
[Multiple cranial neuropathies in a patient with IgG4-related hypertrophic pachymeningitis: a case report].
We describe the case of a 75-year-old woman who presented with acute loss of vision. She experienced subacute headache, hearing loss on the left side, hoarseness, and dysphagia during the previous 10 months. On admission, she had bilateral loss of vision, without any ophthalmological abnormalities, and multiple cranial nerve palsies, including left hearing loss and right IX, X, and XI nerve palsies. ⋯ However, it should be recognized that these conditions might be underdiagnosed. The possibility of central nervous system involvement in IgG4-related disorders should be considered in patients with multiple cranial nerve neuropathies associated with hypertrophic pachymeningitis, even in the absence of systemic sclerosis symptoms. In our case, early treatment with corticosteroids showed immediate effectiveness in correcting the visual symptoms.
-
Familial amyloid polyneuropathy (FAP) is an autosomal dominant genetic disorder with systemic deposition of amyloid fibrils, and is characterized by progressive sensory, motor, and autonomic polyneuropathy. FAP was considered a rare endemic disease; however, its worldwide incidence is much higher than previously recognized. ⋯ Furthermore, a large number of the patients are not good transplant candidates because of their age and/or advanced disease status. Recently, the clinical effects of two transthyretin tetramer stabilizers, diflunisal and tafamidis, were demonstrated in randomized clinical trials, and tafamidis was approved for the treatment of FAP in European countries in 2011 and Japan in 2013.
-
Charcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous group of diseases with over 45 different causative gene mutations identified. Nerve conduction studies are important for the classification and diagnosis of CMT, whereas ultrasound (US) is increasingly used to assess the peripheral nerves of patients with CMT, as a complement to neurophysiological studies. Recent ultrasound assessment reports of peripheral nerves in CMT are summarized here. ⋯ Focal enlargement of nerves at entrapment sites is a characteristic US finding of hereditary neuropathy with liability to pressure palsy. US findings of CMT are thus subtype-specific. Therefore, the assessment of nerve US may become a useful supporting tool for the diagnosis of CMT subtypes.