[Tumor-like inflammatory demyelinating diseases of the central nervous system with relapse onset: a case report and review].

Here we report the clinical, radiological and neuropathological findings of a patient with tumor-like inflammatory demyelinating diseases of the central nervous system.The patient was a 51-year-old man with a four-month history of inflammatory pseudotumor and no other significant medical history, who presented to our hospital recurrent relapse numbness and weakness of his right extremities, dysarthria and memory deterioration. Brain magnetic resonance imaging(MRI) showed mass focal lesion in white matter of left parietal lobes. The biopsy showed numerous infiltrating macrophages and lymphocytes within the perivascular. The patient responded clinically to corticosteroid and intravenous immunoglobulin(IVIG) therapy. According to the results of the biopsy and the MRI , a diagnosis of inflammatory pseudotumor of the central nervous system was made. The vascular dysfunction may act in the pathogenesis of inflammatory pseudotumor of the central nervous system.

[The clinical and pathological characteristics of a patient with glycogen storage disease IV].

OBJECTIVE: To report the clinical and pathological characteristics of one patient with glycogen storage disease IV (Anderson disease). METHODS: The patient was received detailed clinical examinations, ultrasound, electromyography, head MRI and muscle biopsy. RESULTS: The onset of the 22 years old male patient was 7 yrs. The main symptoms were intolerance and fatigue in proximal limbs muscular movement, cardiopalmus by chance. Abdominal ultrasound examinations showed cirrhosis, portal hypertension, splenomegaly. Echocardiogram showed left ventricular myohypertrophia, mild mitral and tricuspid valve insufficiency. Electrophysiology study revealed widespread myogenic changes. Cranial MRI, MRA and MRS were normal. Muscle biopsy showed basophilic intracytoplasmic material in a lot of fibers deposits, which was intensively PAS-positive material and partially resistant to diastase digestion. In the electron microscope, the storage material consisted of filamentous and finely granular material. CONCLUSIONS: There was the first case of glycogen storage disease IV reported in our country, mainly involved skeletal muscle, liver, spleen and cardiac muscle.

[Rheumatoid leptomeningitis: a case report and literature review].

To report the clinical, radiological and neuropathological findings of a patient with rheumatoid meningitis. The patient was a 71-year-old Chinese man with a two-year history of rheumatoid arthritis and no other significant medical history, who presented to our hospital recurrent weakness of his left extremities, dysarthria and a continuous bilateral hand tremor. Cerebrospinal fluid (CSF) and serum examinations were normal apart from a mildly raised serum perinuclear antineutrophil cytoplasmic autoantibody (p-ANCA). Brain magnetic resonance imaging (MRI) showed leptomeningeal enhancement in both frontal and parietal lobes, in addition to several old white matter infarcts. Meningeal biopsy showed numerous infiltrating macrophages and lymphocytes within the leptomeninges. The patient responded clinically and radiologically to corticosteroid and cyclophosphamide therapy. The patient subsequently developed herpes zoster over his left chest as a complication of his immunosuppressive treatment. His cyclophosphamide was ceased and intravenous immunoglobulin (IVIG) therapy was commenced, with good clinical response to both the herpes zoster and meningitis. According to the result of the biopsy, aseptic meningitis was considered the MRI results and the patient's clinical history were given, and a diagnosis of rheumatoid meningitis was made. The patient was p-ANCA positive. Although there was no evidence for cerebral vasculitis on biopsy, it remains a possibility that the patient's recurrent minor cerebral infarcts visible on MRI were vasculitic in nature.