Abnormal spinal MRI findings in human T-cell lymphotrophic virus type I-associated myelopathy

We describe three cases of HTLV-I-associated myelopathy (HAM) with abnormal spinal magnetic resonance imaging (MRI) findings. The serum and cerebrospinal fluid (CSF) of all 3 patients tested positive for anti-HTLV-I antibody. Swelling of the spinal cord with high-intensity lesions on T2-weighted images was observed. Corticosteroid treatment in the case of two patients gradually improved the symptoms, decreased the protein and IgG levels as well as the cell count, and reduced the abnormal MRI findings. To confirm the assumption that abnormal spinal MRI findings are reflective of active inflammation, we compared the clinical parameters, namely, the protein content, IgG level, and cell count, in the CSF of patients exhibiting abnormal spinal MRI findings with those in the case of patients without spinal MRI lesions. The protein contents, IgG levels, and number of cells in the CSF of patients with MRI lesions were significantly higher than those in the CSF of patients without lesions. These findings support the fact that abnormal MRI findings in the spinal cord may be reflective of active inflammation in the early stages of rapidly progressive HAM.     

Cryptococcal meningitis accompanying lymphocytic inflammation predominantly in cerebral deep white matter: A possible manifestation of immune reconstitution inflammatory syndrome

Cryptococcal meningitis is rarely complicated by immune‐mediated leukoencephalopathy, but the precise pathomechanism is uncertain. A 72‐year‐old Japanese man treated with prednisolone for Sweet disease developed a subacute progression of meningitis, which was considered as neuro‐Sweet disease. A treatment by methylprednisolone rapidly improved CSF findings with a remarkable decrease in lymphocyte numbers in the blood, but the patient's consciousness still worsened after the cessation of the treatment. The patient developed cryptococcal meningitis and MRI showed abnormal intensities predominantly in the cerebral deep white matter along with the recovery of lymphocyte numbers in the blood, which resulted in death. A postmortem examination of the brain revealed degenerative lesions, especially at the cerebral white matter and cortex adjacent to the leptomeninges abundantly infiltrated by Cryptococcus neoformans. In the affected cerebral deep white matter, perivascular infiltration of lymphocytes was prominent in coexistence with reactive astrocytes and vascular proliferation, but these findings were not observed in the subcortical and cortical lesions. Cryptococcus neoformans was not present within the brain parenchyma. This is the first report of a case suggesting that cryptococcal meningitis can accompany lymphocytic inflammation predominantly in cerebral deep white matter as a possible manifestation of immune reconstitution inflammatory syndrome.     

Gradient-Echo MRI in Defining the Severity of Cerebral Fat Embolism

Background

A few studies have found that abnormal findings on diffusion-weighted magnetic resonance imaging (MRI) are useful for diagnosing cerebral fat embolism in the acute stage.

Case Report

We applied serial MRI to a case of cerebral fat embolism with cognitive impairment lasting for 2 months. Although marked resolution of the previous abnormal findings was demonstrated, T2*-weighted gradient-echo MRI revealed multiple tiny lesions.

Conclusions

We suggest that T2*-weighted gradient-echo MRI is useful in defining the clinical severity of patients with cerebral fat embolism.     

A case of hypertensive encephalopathy with extensive spinal lesions on MRI

A 14-year-old female had repeated vomiting, headache, abdominal pain, visual field deficit and lethargy at the onset of hypertensive encephalopathy. Cerebrospinal fluid (CSF) test revealed a high level of IgG and protein. MRI demonstrated no supratentorial cerebral lesions but hyperintense lesions were observed from the lower pons to the Th8 level of spinal cord and cerebellar cortex on T2 weighted and FLAIR images without contrast enhancement. The two antihypertensive drugs stabilized to control her blood pressure and improved her clinical symptoms. Reexamination of MRI and cerebrospinal fluid test also revealed clear improvement of the above abnormalities. The abnormal findings on abdominal CT and renography led us to suspect renal infarction. Abdominal angiography demonstrated multifocal stenoses of renal interlobar arteries, which were supposed to supply the renal infarcted regions. These suggested that the renal infarctions due to fibromuscular dysplasia caused systemic hypertension. There have been only two reports that demonstrated spinal cord lesions in reversible posterior leukoencephalopathy syndrome (RPLES). We report extensive spinal lesions on MRI and a high level of IgG in CSF at the subacute phase in a young female with RPLES associated with hypertensive (brainstem) encephalopathy.     

Neuro-Sweet disease: report of the first autopsy case

Background

Neuro‐Sweet disease is a rare condition of central nervous involvement accompanied by cutaneous Sweet lesions. Neuropathological changes in neuro‐Sweet disease are unknown.

Objective

To describe post‐mortem findings of the first case of neuro‐Sweet disease.

Results

A 44‐year‐old Japanese man developed recurrent episodes of cerebral and brainstem encephalitis with cutaneous Sweet lesions from the age of 34 years. His HLA typing was B54 and Cw1, and the symptoms and MRI abnormalities markedly subsided following corticosteroid therapy. Histologically, there were multiple lesions of perivascular cuffing of small venules by macrophages without vasculitis in the thalamus, temporal lobe, basal ganglia, pons, leptomeninges or ventricular ependym.

Conclusions

The core neuropathological findings were: perivascular cuffing around particularly small veins; absence of granulomatous or necrotic angitis; mainly macrophage infiltration; and the thalamus being most affected. In the present case, the diagnosis of neuro‐Sweet disease was made by skin biopsy 5 years after the onset of the central neuron system symptoms. We should pay more attention to skin lesions in steroid responsive recurrent encephalitis in patients who are HLA‐B54 or Cw1 positive.In 1964, Sweet described eight patients with four cardinal features, including fever, peripheral neutrophil leucocytosis, raised painful plaques and a dense dermal infiltration with mature neutrophils.¹ This peculiar “febrile neutrophilic dermatosis” of unknown aetiology, which partly resembles Behçet disease, is currently called Sweet disease. The concurrence of malignancy (haematological neoplasia and various cancers) or systemic inflammatory diseases (inflammatory bowel disease and rheumatoid arthritis) was reported in Sweet disease.² Also, many medicines (granulocyte colony stimulating factor, proteasome inhibitor bortezomib, carbamazepine, etc) were reported as causes of drug induced Sweet syndrome.³ Although it rarely affects the central nervous system, Hisanaga et al^4,5 reviewed cases of Sweet disease with central nervous system involvement, and proposed a concept, “neuro‐Sweet disease”, that is distinct from neuro‐Behçet disease. This paper reports the first autopsy case of neuro‐Sweet disease.     

Clinical presentation and imaging of general paresis due to neurosyphilis in patients negative for human immunodeficiency virus

The clinical presentations and MRI of six patients with general paresis due to neurosyphilis were reviewed. Diagnosis was based on neurological and psychiatric symptoms, positive Treponema pallidum hemagglutination in cerebrospinal fluid (CSF) and sera, and serology that was negative for human immunodeficiency virus by enzyme-linked immunosorbent assay. Most patients had lymphocytic, monocytic pleocytosis and high protein levels in their CSF. One patient had periodic lateral epileptiform discharges, one patient had epileptiform discharges and three patients had slowing of background activity on electroencephalography. Two patients had hyperintense signal abnormalities in the anterior and mesial temporal lobe, while four patients were found by MRI to have cerebral atrophy. Three patients developed white matter lesions. Therefore, clinical, electroencephalography and MRI findings are valuable in the diagnosis of general paresis of neurosyphilis.     

Patient with Gerstmann-Striussler-Scheinker syndrome (GSS P102L) presenting high intensity lesions in the cerebral cortex on diffusion weighted MRI]

A 59-year-old woman with Gerstmann-Str?ussler-Scheinker syndrome (GSS P102L) was reported. She slowly developed progressive gait disturbance and limb ataxia by the age of 58, subsequently followed by dementia and myoclonus. EEG showed periodic synchronous discharges, and MRI by diffusion weighted imaging revealed abnormal high signal intensity lesions in the bilateral cerebral cortex and basal ganglia. A prorin-for-leution substitution at codon 102 of the prion protein gene was demonstrated; and thus, she was diagnosed as GSS (P102L). This is a case of GSS presenting high intensity lesions in the cerebral cortex on diffusion weighted MRI; it suggests that MRI findings disease stages in GSS.     

A case of neoplastic angioendotheliosis--serial study of magnetic resonance imaging

The case of a 59-year-old man who was diagnosed as having neoplastic angioendotheliosis by biopsy of a small hemangioma on the skin is reported. The clinical features were characterized by hypersomnia, memory disturbance, disorientation to time and mild left hemiplegia including the face. Laboratory findings showed an elevated erythrocyte sedimentation rate, increased serum LDH, increased CSF protein and pleocytosis in the CSF. The CSF level of IgG was also elevated and was associated with the appearance of oligoclonal IgG bands. The biopsy specimen of the hemangioma on the skin revealed that some small vessels were packed with atypical mononuclear cells which were positive for anti-B cell antibody. Magnetic resonance imaging (MRI) of the brain detected multiple lesions located in the cerebellum, thalamus and caudate nucleus. The left paramedian thalamic lesion might be responsible for his characteristic mood and behavioral changes. The serial MRI study disclosed that some lesions progressively enlarged and duplicated in number. These findings might be typical for neoplastic angioendotheliosis, in which the rapidly proliferating cells occluded small vessels one after another in the central nervous system. The serial study of MRI may serve an important diagnostic purpose in this disease, although most patients with this disease, so far, have been diagnosed by autopsy.     

An autopsy case of cryptococcal meningoencephalitis: correlation of MRI and pathologic findings

A comparative study of MRI and pathology was performed on a case of cryptococcal meningoencephalitis. An 11-year-old female presented with confusion and vomiting. On admission, CSF examination revealed spherical fungal cells with mild pleocytosis, decreased glucose and elevated protein level. MRI showed multiple punctate lesions in the basal ganglia with high intensity on T2-weighted image, while enhanced MRI revealed diffuse meningeal involvement. Post-mortem examination disclosed that the T2-weighted lesions found in the basal ganglia were aggregated small cystic lesions consisting of a cryptococcal invasion of Virchow-Robin spaces, termed "soap bubble lesions", characteristic findings of cryptococcal meningoencephalitis. Thus MRI findings of the basal ganglia and meninges may help to diagnose cryptococcal meningoencephalitis.     

Hereditary Alzheimer's disease with amyloid angiopathy caused by amyloid precursor protein locus

We report a patient with early-onset autosomal dominant dementia. The CSF showed increased levels of tau protein and decreased amyloid beta (ratio 42:40) typical for Alzheimer's disease. Cerebral MRI revealed vascular lesions and white-matter changes around the posterior horns of the ventricles with only moderate atrophy of the brain. Susceptibility-weighted imaging detected multiple small hemorrhagic changes. Gene analysis revealed amyloid precursor protein (APP) locus duplication as the cause of hereditary Alzheimer's dementia. The co-occurrence of CSF changes typical for Alzheimer's disease and MRI findings of cerebral amyloid angiopathy is remarkable, as it is also described for APP locus duplication. In conjunction with a family history suggestive of hereditary dementia, such a constellation should lead to enhanced gene analysis.     

三例神经梅毒的临床特征与诊断

目的分析神经梅毒的临床特征及提供早期诊断依据。方法回顾性分析经临床和实验室检查确诊的3例神经梅毒患者的有关临床资料。结果神经梅毒的临床特征包括:(1)急性、亚急性起病多,少数慢性起病;(2)临床以间质型,尤其脑卒中最常见;(3)荧光螺旋体抗体吸收(FTA-ABS)试验及梅毒快速血浆反应素试验(RPR)阳性率极高;(4)脑脊液检查表现为蛋白含量增加、细胞数增多(以淋巴细胞为主);(5)头颅CT或MRI表现与高血压、糖尿病所致常见的脑梗死不同,病灶多发,可分布在脑的所有部位。结论神经梅毒误诊率高,临床表现、实验室及影像学检查是其诊断的重要依据。     

A central nervous system lupus showing peculiar findings on cranial magnetic resonance imaging (MRI)]

We report a case of central nervous system (CNS) lupus showing peculiar findings on cranial magnetic resonance imaging (MRI) with remarkable improvement after corticosteriod therapy. The patient was a 28-year-old woman, admitted to our hospital with severe fever, general malaise, and facial edema on June 4, 2001. After admission, she was diagnosed with systemic lupus erythematosus (SLE). On June 6, she showed diplopia at a distance, and on June 10, she suddenly became unconscious and developed general convulsions. Cranial MRI showed asymmetrical, multifocal, high signal intensity lesions on T2-weighted image (T2-WI) and low signal intensity on T1-weighted image (T1-WI). These lesions were primarily present in the subcortical white matter, with some detected in the overlying cerebral cortex. Gadolinium (Gd)-DTPA enhanced T1-WI showed marked leptomeningeal enhancement overlying the lesions on T1-WI and T2-WI. Apparent diffusion coefficient image (ADCI) showed high signal intensity in the surrounding areas of the T1-WI and T2-WI lesions, and low signal intensity in the central areas of the lesions. Diffusion weighted image (DWI) showed high signal intensity in the central areas of the low signal intensity on ADCI. Cerebrospinal fluid (CSF) examination revealed albuminocytologic dissociation (cell counts of 2/microliter and protein level of 108 mg/dl). CSF IgG index was elevated to 1.152 (normal < 0.7) and interleukin-6 (IL-6) activity to 27.2 pg/ml (normal < 4.0). On June 10, Intravenous administration of high-dose methylprednisolone (1,000 mg/day for 3 days) was started to treat CNS lesions of SLE. Her CNS manifestations, CSF findings, and the lesions on the cranial MRI improved remarkably. This is the first case report describing the lesions on both ADCI and DWI in a case of CNS lupus. The findings of ADCI and DWI suggest that the lesions of high signal intensity on ADCI indicate interstitial edema caused by inflammatory microangiopathy, and the lesions of high signal intensity on DWI and low signal intensity on ADCI indicate cytotoxic edema caused by ischemic change resembling microinfarction. We speculate that in addition to usual T1-WI and T2-WI, performing ADCI and DWI is useful for understanding the pathogenesis of CNS lupus lesions, and may play a significant role in the prognosis.     

Diagnostic value of magnetic resonance imaging in multiple sclerosis]

Brain MRI findings in 16 clinical cases of MS were compatible with multiple lesions in the brains in 14 cases. Cerebral lesions were detected in 12 cases without symptoms or signs referable to the cerebral hemispheres. When compared with t*he findings CT scannings, evoked potentials, and oligoclonal bands in the CSF, MRI findings appeared to be the most effective means for confirming the diagnosis of MS, but they would not indicated the course, the times of relapse and the activity of the disease process.     

3例神经梅毒的临床特征分析

目的分析3例神经梅毒的临床特征为早期诊断提供参考。方法回顾性分析3例神经梅毒患者的临床症状、神经影像学和实验室检查特点。结果(1)脑血管梅毒1例,脊髓痨2例;(2)3例血和脑脊液的梅毒抗体均为阳性;脑脊液蛋白含量增加、细胞数增多(以单核细胞为主);(3)脑血管梅毒患者MRA及DSA显示左大脑中动脉M1段闭塞;左大脑前动脉及左大脑后动脉通过皮层支向左大脑中动脉供血区代偿供血;(4)2例脊髓痨患者头颅及胸髓MRI无特殊发现,肌电图及周围神经活检不支持周围神经受损。结论神经梅毒的临床表现与其分型密切相关;首诊易误诊。临床表现及血和脑脊液梅毒抗体阳性是确诊的依据。     

Acute onset of tuberculous meningoencephalitis presenting with symmetric linear lesions in the bilateral thalamus: a case report]

A 18-year-old woman was admitted to our hospital because of high fever and headache. Nuchal stiffness was present, and a CSF examination showed lymphocyte-domonant pleocytosis and a decreased level of glucose. Although antibiotics, aciclovir and an antimycotic drug were administered, disturbance of consciousness, involuntary movements, and pyramidal tract signs appeared. Soon after the medications were changed to antituberculous medicines, the meningoencephalitis started to subside, and was finally cured. Judging from the clinical findings, the CSF findings, the effectiveness of antituberculous medicines, an elevated ADA level in CSF, and positive conversion in tuberculin tests, the final diagnosis was made as tuberculous meningoencephalitis. At the severest stage of the disease, a brain MRI showed symmetric, linear lesions without the effect of Gd-enhancement in the bilateral thalamus, which thereafter disappeared along with the healing of the illness. From all these things, we conclude that thalamic and other parenchymal lesions should be kept in mind in case of acute tuberculous meningoencephalitis.     

A clinico-pathological study of so-called "acute multiple sclerosis" mimicking a brain tumor on the MRI findings]

We are reporting an autopsy case of so-called "acute multiple sclerosis" that was difficult to differentiate from a brain tumor on MRI findings. This case was a 69-year-old man, whose initial symptoms consisted of headache and unsteadiness in walking. Neurological findings included mild ataxia of the left upper extremity and positive Romberg sign. T 2-weighted MRI showed high intensity areas in the posterior limb of the right internal capsule and white matter near the posterior horn of the right lateral ventricle. Although the headache improved, the unsteadiness was exacerbated and the patient became unable to keep standing. Psychiatric symptoms and left hemiparesis were added to the clinical picture. The following MRI proved expansion of the previous lesions and the diffusely enhanced lesion spreading into the contralateral side through the corpus callosum. Stereotaxic biopsy showed the perivascular accumulation of small lymphocytes and a large number of bizarre astrocytes. Primary brain malignant lymphoma was diagnosed and radiation therapy was carried out. However, he developed perforation of the intestinal tract and died. Autopsy findings revealed scattered and disseminated small lesions in the cerebral white matter and the corpus callosum. There were a large number of lipid-laden macrophages, no stainable myelin and preserved axis cylinders in those lesions. Thus, those were interpreted as demyelinting lesions. They were scattered and multiple. This case was radiologically characterised by the diffusely enhanced, expanding butterfly-shaped lesion in bilateral cerebral hemisphere through the corpus callosum, and pathologically proven to be acute demyelination associated with severe perivascular infiltration of inflammatory cells. Multiple sclerosis may mimic neoplastic processes as trans-callosal hyperplastic neuroimage on neuroimaging like the present case.     

Steroid-responsive diffuse cerebral white matter lesions in a case of intractable fungal meningoencephalitis]

We report a case of fungal meningoencephalitis with steroid-responsive diffuse cerebral white matter lesions. A 49-year-old male developed auditory hallucination, confusion and fever, on April, 1994. He was diagnosed as having cryptococcal meningoencephalitis based on the detection of cryptococcal antigens in the cerebrospinal fluid (CSF). Intravenous administration of fluconazole resulted in improvement of his neurologic symptoms and CSF findings. For the next seven months, he was treated with oral fluconazole and the neurological status was stable. However, soon after the dose of fluconazole was tapered, he became confused and febrile, which made him admitted to our hospital. Neurological examination on admission showed disturbance of consciousness, disorientation and meningeal irritation. The CSF examination revealed mild pleocytosis (mostly lymphocytes), elevated protein and normal glucose levels, although fungus was not detected. The T2-weighted image of brain MRI demonstrated diffuse hyperintense lesions in the bilateral cerebral white matters. GD-DTPA enhanced MRI showed spotty enhanced lesions in the periventricular white matters. The neurologic symptoms were once relieved after intravenous administration of fluconazole was started, but two months later, he became comatose and needed ventilatory support, despite amphotericine B therapy. Then, a needle brain biopsy targeting the white matter lesion was done. Histopathology of the specimen showed chronic inflammation with granuloma formation and T lymphycyte infiltrate around the small vessels, though fungus was not detected in the tissue. Combined therapy with corticosteroid and antifungal agents remarkably improved the neurological symptoms as well as the MRI findings. In the present case, fungal infection possibly induced an altered immune reactions which resulted in the steroid responsive diffuse cerebral white matter lesions.     

以痴呆为主要表现的麻痹性痴呆、HIV相关性痴呆和克雅病的临床特征分析

目的探讨并分析梅毒所致麻痹性痴呆(麻痹性痴呆)、HIV相关性痴呆和克雅病等中枢系统感染性疾病所致痴呆的临床特征。方法检索19例患者(麻痹性痴呆8例、HIV相关性痴呆6例、克雅病5例)临床资料,回顾分析其临床表现、实验室检查、脑电图、神经影像学及治疗转归特点。结果 3组患者临床表现均以认知损害为主,并广泛累及多系统(锥体系、锥体外系、小脑)及多组脑神经。辅助检查显示,麻痹性痴呆患者快速血浆反应素环状卡片试验和苍白密螺旋体抗体明胶颗粒凝集试验阳性(8例),脑脊液美国性病研究实验室试验阳性(4例),MRI呈现不同程度脑萎缩(6例);HIV相关性痴呆患者血清HIV抗体筛选试验及Western blotting检测阳性(6例),脑脊液平均蛋白定量明显升高(2例)、潘氏试验阳性(2例),MRI以脑内多发占位病变或大片异常密度影为特征;克雅病患者脑脊液Western blotting检测1433蛋白阳性(4例),脑电波呈弥漫性慢波(4例)伴典型三相波(1例),散发型患者MRI脑叶皮质区沟、回呈肿胀样改变(3例),变异型患者可伴丘脑"曲棍球样"改变(1例)。结论麻痹性痴呆、HIV相关性痴呆及克雅病等中枢系统感染性疾病所致痴呆临床表现复杂多样,诊断时应结合患者病史、实验室血清学和脑脊液指标,以及脑电图和神经影像学表现等综合考虑,明确诊断。     

Encephalo-myelo-radiculitis with high HSV-1 antibody index of CSF

A rare case of encephalo-myelo- radiculitis with high herpes simplex virus type 1 (HSV-1) antibody index of CSF was reported. The case was a 43-year-old male. Double vision, cerebellar ataxia, Th4-12 truncal segmental total sensory disturbance and urinary retention were found, but those symptoms disappeared after 3 weeks of administration of acyclovir and prednisolone. Because the HSV-1 antibody index of the CSF increased significantly, the patient's disease was summarised to have been due to HSV-1, T1-intensified MRI revealed a low-intensity area in the Th5-8 region which coincided with the lesion causing the neurological symptoms, while T2-intensified MRI revealed scattered high intensity area in the cerebral hemisphere. MRI was thus useful for confirming the lesions.     

The impact of magnetic resonance imaging on the management of acute ischemic stroke.

Despite rapid advances in imaging technology, the etiology of stroke remains unestablished in 40% of patients. MRI improves localization in acute stroke. However, it is not known whether "accurate localization" results in better management. We reviewed the hospital records of all patients admitted with a diagnosis of acute ischemic stroke and who had had cranial CTs and MRI within 10 days of admission. Between January 1987 and June 1990, 116 patients (69 men, 47 women; mean age, 66 years) were identified. Compared with CT localization, infarcts were better localized in nine of 39 patients with cerebral cortical lesions, in 20 of 22 patients with brainstem and cerebellar lesions, and in three of three patients with isolated cerebellar lesions. In 22 patients (18.9%), MRI showed multiple infarcts in two or more vascular territories, suggesting embolic disease and leading to anticoagulation. MRI also showed arterial occlusions in 11 patients (9.5%). Based on the information obtained with MRI, the clinical diagnosis was changed in 19 patients (16.3%), resulting in changes in the management of most of those patients. Thus, we confirm earlier reports that MRI improves localization after acute cerebral infarction and show that such information alters patient management.