[A case of Isaacs' syndrome causing various central nervous symptoms successfully treated with high-dose intravenous methylprednisolone therapy].

A 44-year-old man with a bilateral hand tremor suffered from a decline in concentration and abnormal vision for several months. He also complained of easily falling down because of muscle stiffness and cramps in his lower limbs. On admission, he demonstrated lower limb stiffness, muscle cramps, diplopia, hyperhidrosis, left upper limb ataxia and dysesthesia in all limbs. Laboratory examination showed a marked elevation in his serum creatine kinase level (26,890 U/l), and needle electromyography demonstrated myokymic discharges in the muscles of his lower extremities. Isaacs' syndrome was diagnosed based on a positive voltage-gated potassium channel antibody titer of 1,007 pM. Administration of an anticonvulsant (phenytoin, 200 mg/day) did not resolve his symptoms; however, high-dose intravenous methylprednisolone therapy (1 g/day for 3 days) resulted in marked clinical improvement. This case suggests that high-dose intravenous methylprednisolone therapy for Isaacs' syndrome might be as effective as other immunosuppressive therapies such as plasma exchange or intravenous immunoglobulin.

[Overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome].

A 29-year-old female developed diplopia, nasal voice and gait disturbance after an upper respiratory infection. On admission, she presented with bilateral external ophthalmoplegia, slight bilateral facial nerve palsy, dysarthria, dysphagia, cervical and brachial muscle weakness, ataxia and areflexia. She had serum anti-GT1a, anti-GQ1b and anti-galactocerebroside IgG antibodies. She was diagnosed with an overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Intravenous immunoglobulin therapy was effective for the ophthalmoplegia and ataxia, but did not improve the bilateral facial nerve palsy and brachial muscle weakness. The facial nerve palsy clearly worsened despite improvement in other symptoms, and therefore high-dose intravenous methylprednisolone therapy was added. The distinct response to treatment may be caused by different activity, production, clearance and reactivity to intravenous immunoglobulin of the autoantibodies. The present case suggests that treatment response and patterns of recovery differ according to the causative anti-ganglioside antibodies.

Initial MRI findings predict progressive lacunar infarction in the territory of the lenticulostriate artery.

BACKGROUND/AIMS: The mechanisms underlying neurological deterioration in patients with acute lacunar infarction in the lenticulostriate artery (LSA) territory are currently unclear. We aimed to identify predictors for progressive neurological deficits using diffusion-weighted imaging (DWI). METHODS: We studied 40 consecutive patients who were classified into two groups based on their NIHSS scores: progressive infarction (PI) and non-progressive infarction (NPI). We calculated the size of DWI abnormalities and evaluated clinical characteristics on admission for both groups. RESULTS: 19 patients (47.5%) exhibited neurological deterioration. Time from onset to MRI was 8.2 ± 6.7 h for all patients. DWI area was significantly larger in the PI relative to the NPI group (1.1 ± 0.5 cm(2) for PI vs. 0.7 ± 0.3 cm(2) for NPI; p = 0.002), although patients' NIHSS scores at admission were not significantly different between both groups. The optimal cut-off value of infarct area between PI and NPI was 0.98 cm(2). Multivariate analysis revealed that an infarct area on DWI ≥0.98 cm(2) (odds ratio 10.57; 95% confidence interval 2.24-68.32; p = 0.006) was a significant independent predictor of PI. CONCLUSIONS: A large infarct area on initial DWI was an independent predictor of neurological deterioration in patients with acute lacunar infarctions in the LSA territory.

[Fulminant strongyloidiasis successfully treated by subcutaneous ivermectin: an autopsy case].

We report a 49-year-old man who was a human T-cell leukemia virus type 1 (HTLV-1) carrier, born in Okinawa prefecture where both strongyloidiasis and HTLV-1 are endemic. He presented with fever, headache and urinary retention. On the basis of CSF examination and MRI findings, his condition was diagnosed as myelitis. He received methylprednisolone pulse therapy. He was transferred to our hospital due to severe paralytic ileus. Strongyloides stercoralis (S. stercoralis) was found in the duodenal stained tissue of a biopsy specimen. Ivermectin applied both orally and through enema were ineffective because of severe ileus and intestinal bleeding. Nine mg (200 microg/kg) of ivermectin solution was administered subcutaneously every other day for five days (total amount 45 mg). The S. stercoralis burden in the stool decreased and paralytic ileus gradually resolved. Three weeks after the resolution of S. stercoralis infection, purulent meningitis developed and acute obstructive hydrocephalus appeared. The hydrocephalus improved by ventricular drainage. Approximately three months after drainage, he died of incidental aspiratory pneumonia. Autopsy showed neither eggs nor larvae of S. stercoralis in the organs. In this case, the fourth reported case in the world, subcutaneous ivermectin injection was dramatically effective. We should consider a diagnosis of strongyloidiasis for any patient from Okinawa prefecture who was an HTLV-1 carrier presenting with unknown origin ileus after treatment of steroid therapy.