[An autopsy case of amyotrophic lateral sclerosis with prominent muscle cramps, fasciculation, and high titer of anti-voltage gated potassium channel (VGKC) complex antibody].

The patient was a 55-year-old male who had prominent fasciculation and muscle cramps. Muscle weakness and atrophy of the trunk, respiratory system, and extremities gradually progressed. On the basis of these features, we diagnosed this patient as having amyotrophic lateral sclerosis (ALS), however, the upper motor neuron signs were not significant. Following the detection of the anti-voltage gated potassium channel (VGKC) complex antibody at 907.5 pM (normal < 100 pM) and repetitive discharge in a nerve conduction study, immunotherapy with intravenous immunoglobulin, methylprednisolone (mPSL), double filtration plasmapheresis (DFPP), ciclosporin, and rituximab was introduced. mPSL and DFPP showed only tentative effectiveness for fasciculation and muscle cramps, respectively. Thereafter, muscle weakness progressed. The patient died of type II respiratory failure at the age of 57 years, about 2 years after the onset of the disease. At autopsy, a histopathological diagnosis of ALS with lower-motor-predominant degeneration was made. Characteristic cellular features, including Bunina bodies in the remaining lower motor neurons and phosphorylated TAR DNA-binding protein 43-kDa (pTDP-43)-immunopositive inclusions in both upper and lower motor neuron systems, were evident. At present, an immunological role of the anti-VGKC complex antibody in the development of cramp-fasciculation syndrome has been speculated. In this ALS patient, the antibodies might be associated with pathomechanisms underlying the characteristic symptoms.

Coexistence of amyotrophic lateral sclerosis with neuro-Behçet's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient.

We report the clinical and autopsy features of a 65-year-old Japanese man who clinically exhibited overlap of both neuro-Behçet's disease (NBD) and amyotrophic lateral sclerosis (ALS). The patient had a HLA-B51 serotype, a recent history of uveitis and had suffered paraparesis, sensory and autonomic disturbance, frontal signs and tremor. A brain and spine MRI study revealed a longitudinally extensive thoracic cord (Th) lesion, but no apparent intracranial abnormalities. The lesion extended ventrally from Th4 to Th9, exhibiting low intensity on T1-weighted images, high intensity on T2-weighted and fluid-attenuated inversion recovery images and gadolinium enhancement. The patient's upper and lower motor neuron signs and sensory disturbance worsened and he died 16 months after admission. At autopsy, the spinal cord and brain exhibited characteristic histopathological features of both NBD and ALS, including chronic destruction of the ventral thoracic white and gray matter, perivascular lymphocytic infiltration, binucleated neurons, lower and upper motor neuron degeneration, Bunina bodies and skein-like inclusions. Although incidental coexistence of these rare disorders could occur in an individual, this case raises the possibility of a pathomechanistic association between NBD and ALS.

[Case of Lemierre syndrome associated with infectious cavernous sinus thrombosis and septic meningitis].

A 33-year-old man was admitted to our hospital because of right exophthalmos, diplopia and left neck pain. Neurological examination revealed lateral and inferior disturbance of his right eye movement and the meningeal irritation sign. Cerebrospinal fluid showed elevated polynuclear cells. Enhanced CT and MRI revealed thrombophlebitis of the left internal jugular vein and bilateral cavernous sinuses. On the basis of these findings, he was diagnosed as having Lemierre syndrome associated with cavernous sinus thrombophlebitis and bacterial meningitis. After administration of antibiotics, his symptoms disappeared and the data of laboratory analyses also improved. However, after his discharge, he was required re-antibiotics therapy because of septic embolus- induced multiple lung abscesses. Lemierre syndrome is characterized by disseminated abscesses and thrombophlebitis of the internal jugular vein after infection of the oropharynx. Because Lemierre syndrome is potentially life-threatening, early diagnosis and initiation of appropriate therapy are important.

[Guillain-Barré syndrome associated Campylobacter jejuni serogroup Penner A showing antecedent fever and abdominal pain without diarrhea].

We reported a 28-year-old woman who had Guillain-Barré syndrome (GBS). Eight days after fever up and abdominal pain, she had weakness and dysesthesia of both hands and feet. Campylobacter jejuni (C. jejuni) was isolated from her stool culture without diarrhea. IgG anti-GM1 antibody was detected in her serum. Nerve conduction studies revealed a predominantly axonal neuropathy. After intravenous immunoglobulin therapy, her symptoms promptly improved. Penner 19 of C. jejuni was isolated more frequently from the GBS patients than from sporadic enteritis patients. In this case, C. jejuni with the serogroup Penner A was isolated. The serogroup is detected rarely in GBS.

Cerebral infarctions and brain abscess due to Lemierre syndrome.

Lemierre syndrome is characterized by anaerobic bacterial infection in the head and neck, causing thrombophlebitis of the jugular vein and septic metastasis to various distant organs, most often the lungs. Here we describe previously unreported clinical features of cerebral infarctions and brain abscess due to metastatic septic embolism of this uncommon but lethal syndrome, and the successful course of treatment with ampicillin, cefotaxime and metronidazole.