Rinshō shinkeigaku = Clinical neurology
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We experienced a patient of CIDP who was twice complicated with aseptic meningitis following high-dose intravenous gammaglobulin therapy. The patient was a 29-year-old woman who first developed gait disturbance in September 1998. Neurological examination revealed muscle weakness and sensory disturbance in the distal parts of four extremities and decrease of deep tendon reflexes. ⋯ She received high-dose gammaglobulin administrations for 2 days in June 2000 and developed aseptic meningitis again. Over again, 9 months later, she received the same medication for only 1 day in March 2001 and she developed mild headache but not meningitis. Aseptic meningitis with CIDP following high-dose gammaglobulin therapy was rare, however, we should pay attention to this therapy in patients with CIDP and may prevent the occurrence of aseptic meningitis by reducing the total dose and shortening the administration periods of gammaglobulin.
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Carbamoyl phosphate synthetase I deficiency (CPSID) is a rare metabolic disorder affecting the first enzymatic step of urea cycle. We report clinical manifestations of a female case of late-onset CPSID in Japan. An 18-year-old girl was admitted to emergency room due to acute comatose state. ⋯ Genomic DNA analysis showed that she was heterozygous in V1149G mutation. The mutation allele was derived from her father. The causative factor for absence or very low level of maternal CPSI mRNA will require further analysis.
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Case Reports
[A case of Japanese encephalitis presenting with unilateral lesions in diffusion-weighted MRI].
We report the findings of diffusion-weighted MRI (DWI) taken serially in a patient with Japanese encephalitis (JE). The patient was a 43-year-old woman presenting with headache, high fever and consciousness disturbance. The diagnosis of JE was made based on more than fourfold elevation of serum complement fixation antibody titer for JE virus in the convalescent phase of illness. ⋯ The improvement of the DWI findings was paralleled with that of the consciousness level and the cell number and neuron specific enolase concentration in the CSF, suggesting that DWI is useful for evaluation of the disease activity in JE. The lesions in the brain suffering from Japanese encephalitis are usually bilateral and diffuse. To our knowledge, this is the first report of JE presenting with unilateral lesions on MRI, of which phathomechanism remains to be elucidated.
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A 59-year-old man was admitted to our hospital because of right abducens nerve palsy without orbital pain. One month later right orbital pain appeared and two months later the pain changed to an aching severe pain and the right oculomotor, trochlear and trigeminal nerves were also involved. The administration of corticosteroid dramatically reduced these symptoms. ⋯ THS starting with abducens nerve palsy alone is rare, representing only 5.1% of 118 patients. In our patient, the newly developed dynamic MRI was able to reveal the right cavernous sinus lesion. The diagnostic usefulness of dynamic MRI for cavernous sinus lesions has been demonstrated for such cases.
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Review Case Reports
[A case of cephalic tetanus presenting with opisthotonus].
We report a case of cephalic tetanus presenting with opisthotonus. A 49-year-old man was admitted because of repeated convulsions. The patient was a garbage truck driver known to be alcoholic, who fell down to suffer an injury of the left face two days before the onset of convulsion. ⋯ Cephalic tetaus is characterized by a history of an injury of the head and a short latency before developing generalized tonic convulsion or opisthotonus. While a typical case presents with lockjaw, our case presented with opisthotonus, presumably because of early systemic lymphatic spreading of tetanus toxin. Early diagnosis and treatment is important to prevent generalized convulsions which are more frequent and sometimes lethal in cephalic tetanus than the common form.