Meningoencephalitis in relapsing polychondritis: A case report.

RATIONALE: Aseptic meningoencephalitis is a rare central nervous system complication of relapsing polychondritis (RP). PATIENT: We report a 61-year-old Japanese male patient with spiking fever and impaired consciousness. Neurological examination revealed meningealirritation, and cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with elevated protein (199 mg/dL) and interleukin-6 (3810 pg/mL). Serological analysis showed high levels of anti-type II collagen antibodies, and the result of auricular biopsy was consistent with the diagnosis of RP showing cartilage degeneration surrounded by inflammatory cell infiltrations. DIAGNOSIS: A clinical diagnosis of RP was made according to the diagnostic criteria established by MacAdams et al. INTERVENTION: Steroid pulse therapy (methylprednisolone 1000 mg, consecutive 3 days) followed by oral prednisolone (60 mg/day) resolved the patient's high fever and disturbance of consciousness. OUTCOMES: The patient rapidly improved after steroid treatments and has a normal quality of life under the maintenance dose of steroid plus methotrexate (4 mg/week). LESSONS: RP-associated meningoencephalitis is a rare complication with significant morbidity and mortality. It should be considered and differentiated in patients with RP with unexplained spiking fever and impaired consciousness. In addition, the assessment of cerebrospinal fluid interleukin-6 levels may be useful to investigate the disease activity of RP-related meningoencephalitis. Further prospective studies are required to confirm this result.

Diffusion-weighted magnetic resonance imaging in dura mater graft-associated Creutzfeldt-Jakob disease.

PURPOSE: To elucidate the extension patterns of the hyperintense areas on diffusion-weighted magnetic resonance imaging (DW-MRI) in patients with dura mater graft-associated Creutzfeldt-Jakob disease (dCJD). METHODS: We collected the DW-MRI of dCJD cases identified by the CJD Surveillance Committee in Japan, between April 1999 and February 2018. The dCJD cases were classified into non-plaque and plaque-types. The relationship among the abnormal signals, the pathological classification, and the sites of grafting were analyzed. RESULTS: We collected DW-MRI of 11 patients with dCJD, all of whom were methionine homozygous at codon 129 of the prion protein gene. The age at onset was 41 (26-76) [median (range)] years, the age at dural grafting was 19 (10-53) years, and the incubation period was 22 (16-29) years. Eight dCJD cases were classified as non-plaque-type and three cases were plaque-type. Five of the non-plaque-type cases and all the plaque-type cases were pathologically confirmed. Brain DW-MRI was performed 3 (1-22) months after the onset. Most of the non-plaque-type cases showed brighter hyperintensity in the cerebral cortex and basal ganglia on the side of dural grafting. Subsequent DW-MRI showed widespread hyperintense lesions in the brain. Regarding the plaque-type cases, initial scans showed hyperintensity in the basal ganglia and the thalamus in one patient. Another patient's lesion was confined to the basal ganglia. The third patient showed no abnormalities seven months post-onset; however, serial images showed a hyperintensity confined to the thalamus. CONCLUSIONS: Non-plaque and plaque-types demonstrated different patterns of propagation of distinct prion strains.

[Case of primary central nervous system lymphoma after open head injury].

A 65-year-old man had suffered contusion of the left frontal lobe of the brain with a skull base fracture, pneumocephalus, and cerebrospinal fluid (CSF) leakage. He was treated with ceftriaxone 4 g/day for 14 days, but after 1 month he developed multiple cranial nerve palsies (bilateral III-X). CSF contained increased levels of protein (96 mg/dl) and mononuclear cells (72 cells/mm³), and low glucose levels (40 mg/dl, blood sugar 120 mg/dl), but no malignant cells were detected. Magnetic resonance imaging (MRI) disclosed swelling in multiple cranial nerves with Gd enhancement. Anti-biotic and antifungal therapy remitted the facial, glossopharyngeal, and vagus nerve palsies and reduced the Gd-enhancement lesion, as detected by MRI. However, the eyes were bilaterally dilated and medially fixed, and hearing impairments persisted. After 3 months, follow-up MRIs revealed the presence of Gd-enhanced small masses at the ventral pontine base, medulla, and cervicomedullary junctions despite a lack of change in neurological symptoms. Those lesions subsided favorably upon treatment with intravenous and oral corticosteroids. After 1 year and 9 months, Gd-enhanced small cystic masses appeared on the medulla and cerebellum. An open biopsy of the cerebellar tonsillar lesions revealed diffuse large B cell lymphoma. Although the development of primary central nervous system lymphoma after open head injury and infection has not been reported to date, central nervous system lymphomas may mimic diverse neurological diseases. Brain biopsy remains the only definitive diagnosis, and thus should be pursued if blood and CSF markers appear normal.

[A case of hypertrophic pachymeningitis, resolved by antimicrobial therapy].

A 65-year-old woman with diabetes mellitus and chronic otitis media developed headache, fever, and hoarseness, all of which did not responded to the oral antibiotics. As stiff neck and lower cranial nerve palsies appeared, bacterial meningitis was suspected. Neurological examination revealed the right hearing disturbance, right recurrent laryngeal nerve palsy, left sternocleidomastoid muscle atrophy and bilateral tongue atrophy. The CSF examination revealed mild pleocytosis and elevated protein, but no bacterial organism was cultured from the CSF. CT scans showed bilateral mastoiditis, and the right mastoid process and a posterior part of the petrous bone were eroded, indicating the exposed bony structures to the posterior fossa. MRI scans demonstrated the thickening of the dura mater of the posterior fossa and the right cerebellar tentorium. This is a rare example of bacterial pachymeningitis of the posterior fossa, the clinical symptoms and MRI findings of which resolved solely by antimicrobial agents without corticosteroid.

[Dramatic but temporary improvements in a case of CNS intravascular malignant lymphomatosis].

A 54-year-old man with a past history of gastric malignant lymphoma treated by the total gastrectomy and the chemotherapy, developed bilateral sudden deafness one year later. Two years after the gastrectomy he became abruptly paraplegic with sensory impairments of the lower extremities and neurogenic bladder. Serum LDH and soluble IL-2 receptor were high in titers (552 U/l and 1,090 U/l, normal range 145-519). Although the imaging studies of the spinal cord were negative, the myelopathic symptoms resolved dramatically after a course of pulse dose methylprednisolone therapy. However, he soon developed an abnormal behavior and mental deterioration in 3 weeks. The MRIs of the brain revealed abnormal signals compatible with multiple cerebral infarctions. As intravascular malignant lymphomatosis (IML) was suspected because of the laboratory and MRI findings, biopsies of the skin, the bone marrow, the muscle and the lymph node were carried out, without evidence of lymphoma. The brain biopsy ultimately confirmed the presence of IML. The patient remarkably responded to biweekly CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) therapy in terms of regaining the mental alertness and improved hearing. However, the CHOP therapy was prematurely interrupted prior to completion because of infective arthritis. The relapse soon ensued, and he died 6 months after admission. This case was of interest because a solid gastric lymphoma appears to have transformed into the form of intravascular lymphomatosis without mass formations or leukemic changes. Although the neurological symptoms in association with IML are thought to be the results of ischemic events, this case illustrates a remarkable reversibility of the symptoms. This implies that the cerebral symptoms are not necessarily the results of typical ischemic infarction, but due to relative ischemia because of chiefly capillary-venous occlusion by lymphoma cells. The majority of the symptoms is thus attributable to the functional impairment. Therefore, the therapeutic intervention may dramatically improve the symptoms due to IML.

[A case of recurrent bacterial meningitis by delayed cerebrospinal fluid (CSF) leakage due to a head trauma].

A 42-year-old man was admitted due to recurrent bacterial meningitis, as he had been treated here for bacterial meningitis three years prior to the current event. He had a remote history of head injury that he had almost forgotten, and his laboratory data showed no immunodeficiency state. 111In-DTPA cisternography showed an abnormal radioactive accumulation in the frontal lobe adjacent to the left frontal sinus at 23 hours after intrathecal injection, and MPR CT images revealed the left frontal sinus bone fracture. These findings indicated that he had a head injury by which a delayed CSF fistula has been formed. He was surgically treated for a CSF leakage. Although a combination therapy of ABPC and CTRX was efficacious for this patient, this regimen may not be ideal, as meningitis by PRSP has been increasing in incidence. Pneumococcal meningitis, once not a difficult infection to treat, could be a difficult one, as resistant strains to ABPC and CTRX have been more common.

[Marked thickening of the peripheral nerves in chronic inflammatory polyradiculoneuropathy associated with HCV infection].

We report a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a middle-aged patient with hepatitis C virus (HCV) infection. A 49-year-old man presented chronic progressive distal motor weakness and sensory disturbances. Laboratory findings indicated chronic liver dysfunction due to HCV infection, transient positive serum cryoglobulinemia and marked decreased motor and sensory conduction velocities in electrodiagnosis. Lumbar MRI showed marked hypertrophy of the bilateral lumbar radicular nerve roots. Steroid treatment, not IVIg, improved neurological manifestation of this patient, but the serum HCV-RNA level was extremely increased after this treatment. Choice of treatment for patients suffered from CIDP associated with HCV infection is still controversial. Newly discovered patients with HCV infection are increased in Japan in number according to the routine check their anti-HCV antibodies. It is important to monitor carefully the serum HCV-RNA level in CIDP patients associated with HCV infection when steroid treatment is initiated.

[Recurrent multiple cerebral hemorrhages complicated with polyarteritis nodosa].

In January 2001, a 72-year-old woman, who had been treated for polyarteritis nodosa, and vasculitic neuropathy, was admitted in somnolent state. She was monoparetic in her right leg, and disoriented. Neuroimaging studies revealed multiple superficial parenchymal hemorrhages in the bilateral hemispheres with dural Gd enhancement and parenchymal thickenings. No evidence of intracranial vasculitis was seen in visible arterial branches and veins by MRV and helical CTs. While in the hospital, intracerebral hemorrhage recurred three times. In April 2001, the third large intracerebral hemorrhage occurred, for which the removal of hematoma was carried out and brain biopsy was done. The specimens showed an evidence of cerebrovascular amyloidosis, but no inflammation. In view of the absence of angitis in the parenchymal and pial blood vessels, despite the presence of pachymeningitis, the cortical and bridging veins which drained into the venous sinuses, were likely involved in inflammation. Impaired venous drainage due to pachymeningitis might be a mechanism by which recurrent multiple cerebral hemorrhages have taken place in the presence of amyloid angiopathy.