A 23-year-old woman was admitted to our hospital because of consciousness disturbance and convulsion, preceded by high fever, headache and erythema multiforme. Her brain magnetic resonance images showed hyperintense lesions in the left medial temporal lobe and the left pulvinar nucleus of the thalamus on fluid attenuated inversion recovery images. Analysis of cerebrospinal fluid showed mild pleocytosis, but DNA of herpes simplex or herpes type 6 viruses was negative on PCR. Laboratory investigations showed the presence of anti-nuclear antibodies, anti-RNP antibodies and lupus anticoagulant. The clinical diagnosis was made as acute limbic encephalitis associated with SLE, and subsequent administration of prednisolone improved her conditions. In her serum and cerebrospinal fluid, anti-glutamate receptor (ε2, δ2, ζ1) antibodies were detected, and the titers of the antibodies decreased as the symptoms improved. Although the mechanism underlying limbic encephalitis associated with SLE remains unclear, at least in some cases, the anti-glutamate antibodies may play an important role in the pathogenesis of limbic encephalitis with SLE.
Tuberculous meningitis (TbM) is a neurological emergency condition that requires prompt initiation of treatment. The standard initial treatment for TbM is often insufficient for producing remission because the anti-tuberculosis agent may cause severe side effects, or vasculitis and hydrocephalus may induce an intractable state. Moreover, it is difficult to distinguish paradoxical expansion from its own deterioration. We treated 2 cases of adult TbM by using multidisciplinary therapy, including methyl prednisolone pulse and intrathecal isoniazid administration. Both cases had not been diagnosed as pulmonary or other tuberculosis, and cerebrospinal fluid (CSF) culture and polymerase chain reaction at approximately 1 week after hospitalization identified the cases as TbM. We administered the standard initial treatment recommended by the British Infection Society guidelines for adults, but both cases deteriorated and showed elevation of intracranial pressure. We indwelled a lumbar drainage for Case 1 and an Ommaya reservoir for Case 2. We removed CSF and administrated isoniazid regularly using each of the drainage devices, added streptomycin, and increased the steroid dose including addition of steroid pulse therapy. Both cases improved, and their neurological dysfunction did not persist. After the induction of an intractable state occurs due to TbM, we are likely to assume poor prognosis and neurological sequelae. However, our experience in these cases showed amelioration of the symptoms leading to the rehabilitation of these patients in society.
Background: Reticular reflex myoclonus is a rare condition with only a few cases clearly documented on video. The purpose of this paper is to report a patient manifesting typical clinical picture documented on video and characteristic electrophysiological features of reticular reflex myoclonus. Case: A 60-year-old woman presented with spontaneous and stimulus-sensitive myoclonic jerks involving the face, neck and upper extremities following anoxic episode. The patient was investigated electrophysiologically. Surface electromyogram showed brief myoclonic activity starting from the sternocleidomastoid and spreading up to the orbicularis oculi as well as down to the upper limb muscles. Cortical somatosensory evoked potentials and long-latency reflex were not enhanced. Conclusion: Clinical features and electrophysiological findings of this case are consistent with those of reticular reflex myoclonus originally reported by Hallett et al. in 1977.
A 20-year-old previously healthy man presented with prolonged consciousness alteration and severe hypertonia in the extremities after minor head trauma. Laboratory blood tests and cerebrospial fluid (CSF) tests were unremarkable except for an elevated CSF glycine concentratons. Brain MRI revealed hypoplasia of corpus callosum, enlargement of lateral cerebral ventricle and high signal intensity in the bilateral white matter on T2 weighted images. On fluid attenuated inversion recovery images, the signal intensity resembled that of CSF in the central areas of T2 alterations, surrounded by a rim of hyperintensity. These characteristic history and the results of brain MRI and CSF, the diagnosis of vanishing white matter disease (VWMD) was made. VWMD is a rare autosomal recessive leukoencephalopathy which typically begins during infancy or early childhood with a chronic progressive neurological deterioration with cerebellar ataxia and spasticity. Recently, milder variants of the disease with adult onset have been reported. VWMD should be included in the differential diagnosis of leucoencephalopathy in young adults.
Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by sudden onset of headaches, visual disorders, decreased consciousness, and convulsion associated with brain edema occurring in the occipital lobe. Several different causes including malignant hypertension, eclampsia, renal failure, and use of immunosuppressants have been reported in patients with RPLS. Our patient was a 45-year-old man who presented with fever, arthralgia, and melena approximately 1 year previously and received the diagnosis of Wegener's granulomatosis. Following steroid therapy his symptoms ameliorated; however, during the course of the illness he developed tension-type headache, nausea and vomiting, and bilateral loss of visual acuity. On admission, his visual acuity was markedly decreased without any abnormal findings in the optic fundus. There was no neurological deficit except the visual symptoms. Imaging of the head revealed multiple lesions in the white and gray matter of the bilateral occipital lobe and cerebellar hemisphere, which proved vasogenic edematous lesions by the hyperintense signals in T2 weighted, FLAIR, and diffusion weighted images, suggesting the diagnosis of RPLS. Treatment with antihypertensive drug and glycerol was initiated and the patient made a full clinical recovery within a few days. The pathogenesis of RPLS is not fully understood. Our case was not on any immunosuppressant therapy at the time of onset of RPLS, and his hypertension was mild and transient without renal failure. It is possible that RPLS in our patient might be a manifestation related to Wegener's granulomatosis-mediated vascular endothelial injury.
We report a 30-year-old man with recurrent eosinophilic encephalomyelitis. He had a history of childhood asthma and allergic rhinitis. A half year before admission, when he suffered from a headache, a few lesions were indicated by brain MRI at another hospital. From a month before admission, he noticed gait disturbance, sensory impairment, difficulty in micturition, and constipation. Neurological examination revealed moderate muscle weakness in the feet, hypoesthesia below Th6, and bladder-bowel disturbance including impotence. Lumbar T2 weigthed MRI showed a severe swelling and a hyperintense lesion at the conus medullaris. Brain MRI revealed several asymptomatic white matter lesions. Eosinophilia was documented in the cerebrospinal fluid (CSF) but not in the peripheral blood. Clinical symptoms and MRI findings were remarkably improved after steroid pulse therapy. Note that eosinophils in the CSF were also decreased after the treatment with apoptosis-like cells. We thought that CSF eosinophilia was the core pathogenic feature of this case, but clinical settings that provoke CSF eosinophilia such as parasites and other infectious agents, neuromyelitis optica, atopic myelitis, eosinophilic leukemia and hypereosinophilic syndrome could be ruled out. The remarkable responses to steroids without any additional therapy, compatible with idiopathic eosinophilic syndromes, confirmed that this was a case of idiopathic eosinophilic recurrent encephalomyelitis.
We experienced a patient with cat-scratch disease (CSD) who developed encephalopathy. The subject was a 34-year-old female who had been aware of a low-grade fever and swollen inguinal lymph nodes for 1 month. The subject's consciousness became impaired after a headache developed, accompanied by fever, nausea, and vomiting, and she subsequently sought medical consultation. No major abnormalities were observed in the cerebrospinal fluid and cranial magnetic resonance imaging (MRI) was normal. However, necrotizing lymphadenitis was observed on contrast enhanced computed tomography (CT) of the pelvis and granulomatous changes were observed in a surgical lymph node biopsy. As the subject lived with a pet cat, PCR testing for Bartonella henselae (the CSD pathogen) was performed using a tissue biopsy. This was positive and the subject was diagnosed with CSD encephalopathy. There are very few domestic reports of CSD encephalopathy and care must be taken not to overlook this disease.
A 53-year-old woman visited us for a neurological consultation before her liver transplantation. She had a history of primary biliary cirrhosis that began at 37 years of age. She showed falling episodes and met with a traffic accident at 52 years old. Since then, her symptoms had worsened. The neurological examination showed masked face, rigidity on bilateral arms and legs, and lack of balance. Her Mini-Mental State Examination Score was 28/30, but she suffered from loss of memory and had trouble with executive function in detailed examinations. Her T1 weighted image showed hyperintensity in bilateral globus pallidus, putamen, dentate nucleus and cerebral peduncle. There was a significant improvement in intellectual function and neurological signs 6 months after her orthotopic liver transplantation. In addition, post-liver transplantation images showed a decrease in the area of hyperintensities. This case suggests that even in a patient with severe liver cirrhosis a complete cure of neurological manifestations can be obtained after the liver transplantation.
An 86-year-old woman was admitted following generalized seizure. Postictally she showed disturbance of consciousness, right hemiparesis, and right spatial neglect. Brain fluid attenuated inversion recovery (FLAIR) imaging demonstrated mainly left-sided, but asymmetrical, subcortical white matter lesions. On the second day, level of consciousness improved, along with right hemiparesis and right spatial neglect. Electroencephalography displayed mainly α waves and sporadic θ waves without sharp waves. One week later, however, the patient again experienced somnolence and right hemiparesis. FLAIR revealed day-by-day enlargement of white matter lesions. The possibilities of hypoglycemia, hyperammonemia, hypothyroidism, Hashimoto encephalopathy, collagen disease, antineutrophil cytoplasmic antibody-related angiitis, and infection were excluded based on the results of blood biochemistry and cerebrospinal fluid. We initially suspected intravascular lymphoma, so random skin biopsy was performed, but the results were negative. We then suspected cerebral amyloid angiopathy because of the presence of dementia and multiple microbleeds on T2* weighted magnetic resonance imaging. Cerebral biopsy revealed amyloid deposition in cortical arterioles and CD3-positive T cells in the perivascular space. Cerebral amyloid angiopathy-related leukodystrophy was therefore diagnosed and immunosuppressive treatment was started. After 14 days of treatment, clinical symptoms and results of FLAIR imaging were significantly improved. When patients display asymmetrical subcortical white matter lesions with microbleeds on T2* weighted imaging, amyloid angiopathy-related inflammation should be considered.
A 62-year-old woman had progressive dysarthria for 2 months and was suspected of having amyotrophic lateral sclerosis because of the presentation of bilateral tongue atrophy and fasciculation. Brain magnetic resonance imaging (MRI) showed enlargement of the left hypoglossal nerve, and whole-body gallium scintigraphy showed abnormal uptake in the left pelvic cavity and left thigh. On the basis of the findings of biopsy of the mass lesion in the left thigh, she was diagnosed with diffuse large B-cell lymphoma. After chemotherapy for diffuse large B-cell lymphoma, the tongue atrophy improved. The patient subsequently developed left oculomotor nerve palsy, weakness of the right arm, and weakness of the right leg. The cause of these symptoms was thought to be neurolymphomatosis on the basis of the typical MRI findings observed. We report a rare case of neurolymphomatosis presenting as bilateral tongue atrophy, mimicking amyotrophic lateral sclerosis.