We report a case of IgM paraproteinemic neuropathy associated with anti-sulfated glucuronic paragloboside (SGPG) IgG antibody. An 84-year old man complained of numbness on the left side of the face and in the distal portions of the limbs. Neurological examination showed mild sensory ataxia. The laboratory tests revealed the presence of IgM lambda paraproteinemia and anti-SGPG IgG antibody without anti-myelin-associated glycoprotein (MAG) activity and anti-MAG/SGPG IgM antibody. Results of nerve conduction study showed decreased sensory nerve action potential (SNAP) amplitude, indicating the presence of sensory-dominant axonal polyneuropathy, and the prolongation of distal latency was not observed. Treatment with corticosteroids resulted in a rapid improvement in neurological abnormalities. In IgM paraproteinemic neuropathy associated with anti-MAG/SGPG antibody, distal acquired demyelinating sensory neuropathy and resistance to immunological treatments are the characteristic pathologic and clinical features, respectively. On the other hand our rare case of IgM paraproteinemic neuropathy positive for anti-SGPG IgG antibody presented with axonal sensory polyneuropathy and a good responsiveness to corticosteroids.
A 49-year-old man was admitted to our hospital with progressive gait disturbance. Our examination revealed a low grade fever, weight loss derived muscle weakness, sensory disturbance and loss of deep tendon reflex of the lower extremities. Magnetic resonance imaging (MRI) detected an abnormal intensity and gadolinium enhancement in the cauda equina. Two weeks after admission, disturbance of consciousness and bladder appeared. Cerebrospinal fluid examination showed pleocytosis, elevated protein and soluble IL-2R, but cytological examination was class II negative. We performed a cauda equina biopsy urgently and diagnosed malignant lymphoma, of a diffuse large B-cell type. We selected combined MTX-based chemoradiotherapy and his symptoms significantly improved after a month. He achieved complete remission and remains recurrence-free after 10 months post treatment although he remains with light paraparesis and sensory disturbance of the lower extremities. He has already gone back to a normal life. An examination of cauda equina biopsy led to quick diagnosis and treatment.
A 16-year-old woman with MELAS developed fever and myoclonic epilepsy which improved with conventional anti-epileptic drugs. Since seizures recurred one month after successful treatment, the doses of phenobarbital, clonazepan, and valproate were increased. However, there was no improvement and status epilepticus continued. The addition of lamotrigine resulted in a decreased frequency and good control of seizures. This case is important, showing satisfactory results from the addition of lamotrigine for treatment-resistant status epilepticus.
A right-handed Japanese man developed memory loss at 51 years of age. The right side clumsiness developed from 52 years of age and then progressively worsened. Temporal/parietal lobe atrophy was observed predominantly on the left side upon MR imaging. Subsequently, limb-kinetic apraxia and parkinsonism became apparent predominantly on the right side. These symptoms became aggravated along with dementia, ultimately leading to an apallic state. The patient eventually died at the age of 59 due to aspiration pneumonia. An autopsy was carried out and cerebral atrophy was observed predominantly on the left side. Senile plaques were observed on the entire cerebral cortex at a high frequency, along with many cotton wool plaques. Anti-phosphorylated tau-positive neurofibrillary tangles and several neuropil threads were observed upon immunostaining. The tau-positive structures were also positive for both RD3 and RD4 antibodies. The findings of tauopathy of the glia were poor, and the tau lesion of the brainstem was milder than that of the cerebral cortex. These results suggest the possibility that the corticobasal syndrome clinically developed in some type of Alzheimer’s disease and a definite diagnosis was made only by pathological examination.
A 50-year-old woman presented with constriction sensation in the lower part of the chest, paresthesia in the right forearm and hypesthesia in the right thigh. One month later, she noticed a loss of sense of smell. The normal findings of the nasal mucosa and the impaired results of standard olfactory acuity test (T&T olfactometry) and intravenous olfactory test (Alinamin test) suggested a lesion proximal to the nasal mucosa. Sensory disturbances in the segmental areas of cervical, thoracic and lumbar regions were clinically and electrophysiologically attributed to polyradiculopathy. MRI of the brain and whole spine revealed no abnormalities. Cerebrospinal fluid examination showed lymphocytic pleocytosis. Sarcoidosis was diagnosed based on the findings including an elevated serum angiotensin-converting enzyme level, bilateral hilar lymphadenopathy on the chest CT and histological evidence of noncaseating granulomas. Methylprednisolone pulse therapy improved the olfactory and sensory disturbances. Neurosarcoidosis should be considered in the differential diagnosis of olfactory impairment.
A-61-year old man was admitted to our hospital with fever and severe disturbance of consciousness. He was diagnosed with Streptococcus pneumoniae meningitis on the basis of cerebrospinal fluid (CSF) analysis and urinary antigen detection by immunochromatography. Although he was treated with dexamethasone and antibiotics, his general status worsened as systemic inflammatory response syndrome (SIRS), disseminated intravascular coagulation (DIC), and status epileptics developed. Following treatment with polymyxin B-immobilized fiber therapy (PMX), which can also absorb bacteria-derived toxic substances, he recovered from DIC and SIRS, and disturbance of consciousness improved immediately. In addition, the concentration of several CSF cytokines—IL-1, IL-2, and TNF-α—was decreased. The present case suggests that PMX is a good option for severe bacterial meningitis.
A 66-year-old man presented with deteriorated bradykinesia, gait disturbance, disorientation, and urinary incontinence for three weeks. Magnetic resonance imaging (MRI) showed dilatation of the ventricles. Cerebrospinal fluid (CSF) examination demonstrated lymphocytic pleocytosis, elevation of protein levels, and decreased of glucose levels. A gadolinium-enhanced MRI revealed lesions in the ventricular wall and choroid plexus, mimicking ventriculitis. No evidence of bacterial, fungal, mycobacterial, or viral infections were observed in the CSF. Flow cytometry of CSF showed predominance of CD20+, λ+ cells. PCR examination of CSF revealed positive IgH gene rearrangement, suggesting B cell lymphoma. Endoscopic brain biopsy showed diffuse large B cell lymphoma. As the patient had no evidence of lymphoma in the other organs, we made a diagnosed of primary central nervous system lymphoma (PCNSL). A limited intraventricular spread of PCNSL is rare but important as one of differential diagnosis of ventriculitis.
We report the case of a 33-year-old man with diplopia, sleepiness, and paresthesia of the left upper limb that were slowly progressive. On admission, he presented with restriction in the vertical movement of the eyes and abduction of the right eye, and horizontal and convergence nystagmus. Slight weakness of the left upper limb, bilateral Babinski sign, and truncal ataxia were also noted. Cerebral magnetic resonance imaging was performed, and gadolinium-enhanced T1-weighted imaging revealed a mass lesion that involved the diencephalon and the corpus callosum, which was invariably enhanced. Specimens obtained using a brain biopsy showed epithelioid granuloma with the presence of foreign body giant cells and lymphocytic infiltration. Prednisolone was administrated because we suspected neurosarcoidosis, but the clinical symptoms worsened with the enlargement of the lesion. A re-evaluation of the biopsy specimens using immunohistochemistry revealed tumor cells of germinoma that were scattered among the lymphocytes and positive for periodic acid-Schiff staining, placental alkaline phosphatase, and c-kit. A combination of chemotherapy and radiation resulted in clinical improvement and marked reduction of the mass lesion in size. We concluded that the possibility of germinoma should be considered in case granulomatous inflammation is observed in brain biopsy specimens.
A 32-year-old-immunologically healthy woman suffered from mumps. A few days later, she was brought to our hospital because of generalized convulsions. On arrival, she developed a decorticate posture. Anti-mumps virus antibodies were detected in her serum. Elevated protein levels without pleocytosis were observed in her cerebrospinal fluid. On the basis of a diagnosis of mumps-associated encephalitis/encephalopathy, steroid pulse therapy was administered, which improve her disturbance of consciousness. She exhibited akinetic mutism, which was followed by deterioration of the frontal lobe. Single-photon emission computed tomography demonstrated decreased regional cerebral blood flow in the bilateral frontal regions. Therefore, she was suspected with frontal lobe dysfunction associated with the lesion, including the thalamus and/or brain stem. We consider that the encephalitis/encephalopathy present in this case was caused by a reversible autoimmune process triggered by mumps virus infection.
A 57-year-old woman presented with motor and cognitive impairments under treatment for cryptogenic organizing pneumonia with immunosuppressive agents. Magnetic resonance imaging showed widespread signal abnormalities in the cerebral white matter. Susceptibility-weighted imaging showed attenuated contrast of the cerebral medullary vein in the lesions, and 18F-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) revealed decreased uptake at the same site and increased uptake in multifocal lung involvements. Lung biopsy findings were consistent with diffuse large B-cell lymphoma. Polymerase chain reaction for JC Virus DNA in cerebrospinal fluid yielded positive results. Based on these findings, the present case was given a diagnosis of progressive multifocal leukoencephalopathy (PML). The patient was treated with oral mefloquine, but her respiratory condition deteriorated and chemotherapy was required to prevent further deterioration. As a result, chemotherapy to treat lymphoma could not result in beneficial immune reconstitution, PML continued to progress despite mefloquine treatment, and the patient developed decorticate posture. The efficacy of mefloquine in patients with non-HIV-associated PML warrants further investigation.