Nobuya Fujita

A case of Behçet disease developing recurrent ischemic stroke with fever and scrotal ulcers

A 30-year-old man, who was diagnosed with Behçet disease at 10 years of age, was hospitalized because of transient right hemiparesis after presenting with high fever and scrotal ulcers. Brain MRI revealed ischemic lesions in the area supplied by the anterior cerebral arteries. Analysis of cerebrospinal fluid (CSF) showed pleocytosis and a high interleukin-6 (IL-6) concentration (668 pg/ml). The patient was diagnosed with acute ischemic stroke associated with exacerbation of Behçet disease. After initiation of corticosteroid therapy, his clinical symptoms improved, and the CSF IL-6 concentration decreased. One year later, the patient developed high fever and scrotal ulcers after the onset of transient left upper limb plegia. Brain MRI showed an acute ischemic lesion in the right putamen, and CSF analysis showed an elevated IL-6 concentration (287 pg/ml). Brain CT angiography revealed stenosis of the left anterior cerebral artery and occlusion of the right anterior cerebral artery, which had been well visualized one year previously. Involvement of the intracranial cerebral arteries in Behçet disease is extremely rare. To the best of our knowledge, this is the first case report of a patient with recurrent symptomatic ischemic stroke associated with high fever and scrotal ulcers, which suggests exacerbation of Behçet disease.

A case of multiple cerebral hemorrhage caused by sudden increase of eosinophil in a patient with eosinophilic granulomatosis with polyangiitis

A 42-year-old woman with bronchial asthma was admitted to our hospital due to sensory dominant mononeuritis multiplex lasting for more than 6 months. At that time, her eosinophil count was 761/μl and her sural nerve biopsy showed no findings suggestive of vasculitis. Four months later, she experienced sudden convulsions and right hemiparesis due to left lobular parietal subcortical hemorrhage, when her eosinophil count was elevated to 3,257/μl. Numerous microbleeds and small infarctions were also detected in the intracerebral areas of different regions with MRI. Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis of the small vessels, commonly affecting the peripheral nerves. Subarachnoid hemorrhage in patients with EGPA is extremely rare. Steep elevation of the eosinophil count may release certain cytokines, causing cerebral hemorrhage.

Herpes simplex encephalitis presenting as stroke-like symptoms with atypical MRI findings and lacking cerebrospinal fluid pleocytosis

A 73-year-old woman presented with sudden onset of right hemiparesis and was diagnosed as having cerebral infarction on the basis of diffusion-weighted brain MRI, which demonstrated lesions in the left parietal cortex. On the 3rd day, the patient developed right upper limb myoclonus, aphasia, and disturbance of consciousness with high fever. On the 6th day, she was transferred to our hospital with suspected viral encephalitis, and treatment with acyclovir was started. By the 6th day, the lesions detected by MRI had expanded to the gyrus cinguli, insula and thalamus, but not to the temporal lobe. At that time, the CSF cell count was 8/μl, and this later increased to 17/μl by the 13th day. Although herpes simplex virus DNA was detected in the CSF on the 6th day, there was no evidence of CSF pleocytosis or temporal lobe abnormalities demonstrable by brain MRI throughout the whole follow-up period. This was very atypical case of herpes simplex encephalitis characterized by a stroke-like episode, atypical MRI findings, and absence of cerebrospinal fluid pleocytosis. It is important to be mindful that herpes simplex encephalitis (HSE) can have an atypical presentation, and that sufficient acyclovir treatment should be initiated until HSE can be ruled out.

Adult-onset adrenoleukodystrophy of the olivopontocerebellar form and lacking cerebral involvement

A 40‐year‐old man presented with subacute progressive gait disturbance. Two uncles on his mothers side were diagnosed previously as having adult‐onset adrenoleukodystrophy. Fluid‐attenuated inversion recovery magnetic resonance imaging showed hyperintense regions in the cerebellum, middle cerebellar peduncles, pons and midbrain with gadolinium enhancement without cerebral involvement. A missense mutation of the ABCD1 gene (c.1661 G>A) was found. We should note that adrenoleukodystrophy of the olivopontocerebellar form can be present, even if there is no involvement in the cerebrum. This case is unique, because it showed bilateral pontocerebellar pathway involvements symmetrically. If patients with the olivopontocerebellar form are diagnosed early, it might be possible to suppress the progression of the disease with bone marrow transplantation.

[A case of conus medullaris infarction expanding to the vertebral bodies, major psoas and erector spinae muscles].

A 77-year-old woman presented with conus medullaris and cauda equina syndrome following a sudden pain in the bilateral lower abdomen and right buttock. Lumbar magnetic resonance imaging (MRI) showed not only a conus medullaris lesion, but also several lesions in the vertebral bodies (L1, L2), right major psoas muscle, right multifidus muscle and bilateral erector spinae muscles. As these areas receive blood supply from each branch of the same segmental artery, we considered all of the lesions as infarctions that were a result of a single parent vessel occlusion. It is known that a vertebral body lesion can be accompanied by a spinal cord infarction, but in combination with infarction of a muscle has not been reported. This is the first report of a concomitant spinal cord and muscle infarction revealed by MRI. It is noteworthy that a spinal cord infarction could expand not only to neighboring vertebral bodies, but also to muscles.

[Transformation from chronic subdural hematoma into subdural empyema following cat bites: a case report].

A 69-year-old man developed motor aphasia and right hemiparesis with severe headache, during the treatment of cellulitis and sepsis due to cat bites. Brain CT showed a low density, crescent-shaped lesion in the left subdural space, which was hypointense on brain diffusion-weighted imaging (DWI). One week later, when his neurological symptoms had worsened, the signal of the subdural lesion had changed to hyperintense on DWI. The lesion was capsule-shaped when enhanced by Gadolinium. The signal changes on DWI of the lesion indicated the existing hematoma had changed to an empyema, or so-called infected subdural hematoma, due to a hematogenous bacterial infection. Pasteurella multocida, a resident microbe in the oral cavity of cats, could be the responsible pathogen in this case. The patient recovered completely after treatment with intravenous high dose antibiotics. This is an important case report describing the transformation from a chronic subdural hematoma into a subdural empyema by DWI.