Resolving MRI lesions in lupus erythematosus selectively involving the brainstem

An adolescent girl with systemic lupus erythematosus presented with selective brainstem dysfunction as the initial sign of central nervous system involvement. Although computed tomography was initially normal, magnetic resonance imaging demonstrated multiple, large brainstem lesions. Serial magnetic resonance imaging, which revealed resolution of most of these lesions, correlated with a good clinical outcome. We postulate that this magnetic resonance imaging pattern represents microinfarction with surrounding edema.     

Multiple sclerosis with sensory disturbances and hyperhidrosis of the hand

INTRODUCTION: Hyperhidrosis may result from brain, spinal cord or peripheral nervous system injuries. We report the case of a 26-year-old patient who presented with sensorial disorders and focal hyperhidrosis of the right hand. OBSERVATION: MRI revealed multiple lesions very suggestive of active demyelinization. A lesion in the left paramedian posterior portion of the pons was enhanced after gadolinium infusion. The diagnosis of multiple sclerosis was suggested by MRI data and oligoclonal IgG bands on cerebrospinal fluid electrophoresis. CONCLUSIONS: Focal hyperhidrosis may be due to a lesion of the crossed sympathetic inhibitory tract. Although autonomic dysfunction is common during the late course of multiple sclerosis this case is, to our knowledge, the first report of focal hyperhidrosis revealing multiple sclerosis.     

Kombiniertes Auftreten von amyotropher Lateralsklerose und Multipler Sklerose

The concurrence of amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) is extremely rare. We report on a 55-year-old female patient presenting with a progressive gait disorder for 6 months and a speech disorder for 3 months. Neurological examination revealed a spastic paraparesis and mild dysarthria and dysphagia. Technical and laboratory investigations met the diagnostic criteria for MS: magnetic resonance imaging showed multiple periventricular white matter and cervical lesions; cerebrospinal fluid showed a typical autoimmune response. Within the following 3 months generalized fasciculations, atrophy of the small hand muscles and bulbar signs were noticed. Nerve conduction studies revealed acute and chronic signs of denervation in all limbs without nerve conduction block. Hence clinical and paraclinical examination met the El Escorial criteria for ALS. Although myelitic lesions in the anterior horn cells may lead to peripheral segmental denervation, the generalized denervation suggested the unusual coincident combination of ALS and MS in this patient. In clinical praxis motoneuron diseases should also be considered in patients with pronounced peripheral denervations once "definite" MS has been diagnosed.     

Periodic lateralized epileptiform discharges (PLEDs): a possible role for metabolic factors in pathogenesis

Periodic, lateralized, epileptiform discharges (PLEDs) in the electroencephalogram have been reported in association with a variety of structural lesions of the brain. Although a great deal of attention has been directed to the neuropathological basis of PLEDs, little emphasis has been placed on the functional basis of this EEG syndrome. In this paper, we reported a patient with multiple systemic problems whose EEG showed PLEDs. However, radiological and pathological studies revealed no neuropathological basis for the occurrence of this EEG syndrome. Thus, we presented evidence that metabolic factors may play a part in the pathogenesis of PLEDs.     

Multiple cystic brain lesions in a patient with pilocytic astrocytoma.

Pilocytic astrocytomas are usually present as solitary posterior cranial fossa tumours. An unusual case of pilocytic astrocytoma in a 3 year and 8 month old boy is presented. The patient presented over the course of 10 months with intermittent headaches, vomiting, gait ataxia and drowsiness. After extensive investigations magnetic resonance imaging (MRI) revealed widespread lesions throughout the central nervous system, including multiple cystic cerebral grey matter lesions. A brain biopsy was performed and pathological studies revealed pilocytic astrocytoma. The literature pertaining to neuraxis dissemination of pilocytic astrocytomas in the paediatric population is reviewed. There are only a very limited number of reports of pilocytic astrocytoma ca using multiple brain lesions, with no publications of multiple cystic brain lesions. We believe this to be a unique case of pilocytic astrocytoma presenting with widespread cystic lesions throughout the brain.     

Primäres ZNS-Lymphom während der Behandlung von Autoimmunerkrankungen mit Azathioprin

We present a 31-year-old female patient with primary non-Hodgkin's lymphoma of the CNS after immunosuppressive therapy. Colitis ulcerosa had been diagnosed 2 years previously. Prophylactic therapy with azathioprine over 9 months was stopped after the development of listeria meningitis which was treated successfully with antibiotics. At this time native CCT was normal. Three months later the patient developed an epileptic seizure and multiple cerebral lesions were detected in CCT and MRI. Although antibiotic therapy was started, the cerebral lesions showed no regression. Stereotactic biopsy revealed immunochemical and histologic high-grade malignant B cell lymphoma. The risk of primary CNS lymphoma under azathioprine treatment for an autoimmune disease with a possible congenital immunodeficiency is presented and the literature is reviewed.     

Systemic Rosai–Dorfman disease with central nervous system involvement

Rosai–Dorfman disease (RDD) is a rare idiopathic and lymphoproliferative disorder. Central nervous system (CNS) involvement is infrequent and typically manifests as an isolated lesion. In this article, we describe an unusual case of RDD with multiple lesions in the CNS, upper respiratory tract and lymph nodes. A literature review revealed 45 cases (including the one described herein) of systemic RDD with CNS involvement documented to date. Among these cases, 29 (64.4%) presented with intracranial lesions, 10 (22.2%) with spinal lesions and 6 (13.3%) with both. While the condition of most patients was stable, only four died from RDD or for other reasons. Prognosis is good for most patients, even those with extensive lesions. Although there are various treatments for RDD, surgery is preferred. Given the rarity of RDD, multicenter international collaborations are advocated in order to study disease pathogenesis and develop effective treatment strategies.     

A case of primary central nervous system malignant lymphoma developing from the optic chiasma and hypothalamus]

A 63-year-old woman was admitted to our hospital with a 1-month history of visual field defect and reduced bilateral visual acuity. Neurological examination revealed enlargement of bilateral central scotoma and blurred vision, and brain MRI showed masses in the optic chiasma and right hypothalamus on Ti-weighted image with contrast enhancement. Although the patient was treated with high-doses of methylprednisolone four times, the masses spread to the right internal capsule and the vicinity of the right lateral ventricle. An open biopsy was performed and microscopic examination of the sample revealed lymphoplasmacytic lymphoma. Although primary central nervous system lymphoma (PCNSL) developing from the optic chiasma and hypothalamus is rare, PCNSL should be considered in patients with multiple lesions in the optic chiasma and hypothalamus.     

Multifocal malignant optic glioma of adulthood presenting as acute anterior optic neuropathy

We report a 63-year-old, previously healthy female patient with glioblastoma multiforme of the optic nerve and chiasm presenting as acute anterior optic neuropathy. She presented with a 3-week history of progressively increasing headaches, retrobulbar pain, rapidly progressive visual loss in the right eye and blurred vision in the left eye. Early clinical examination revealed right optic disc swelling and she was initially diagnosed with demyelinating optic neuritis. Her clinical course deteriorated with total visual loss in the right eye and progressive visual loss in the left eye despite treatment with intravenous (IV) methylprednisone and IV immunoglobulins. MRI revealed enhancement of the right optic nerve and optic chiasm, with multiple periventricular hyperintense foci. Six weeks later, the patient presented with left facial palsy and left hemiparesis. Follow-up MRI showed multiple enhancing lesions in addition to the previous lesions involving right lentiform and right thalamic nucleus, right cerebral peduncle, right temporal and parietal lobes. Although the optic nerve biopsy was inconclusive, the brain biopsy revealed glioblastoma multiforme. This report demonstrated that malignant glioma of adulthood may be multicentric and may mimic optic neuritis clinically, which might help explain the difficulties in diagnosis.     

Multiple cerebral white matter lesions following head trauma with eyeball contusion]

We reported a 36-year-old man with multiple cerebral white matter lesions following head trauma with eyeball contusion. He had suffered from optic neuritis on non-injured side after one and half months from the head trauma. Brain MRI revealed multiple cerebral white matter lesions and lumbar puncture disclosed an elevated level of protein of the cerebrospinal fluid after two and half months from the head trauma. He was treated with steroid pulse therapy and resulted in an improvement of his visual acuity and a remarkable decrease of multiple cerebral white matter lesions. There has been a controversy concerning the causal relationship between trauma and multiple sclerosis (MS). In this case, MS-like multiple cerebral white matter lesions are considered to be relevant to the head trauma.     

Atypical psychosis with disseminated subpial demyelination

A 34-year-old woman experienced three episodes of an atypical psychosis, characterized by confusion, agitation, delusional thinking, paranoid ideation, and auditory hallucinations, during the 14 months prior to her death. Findings of gross examination of the brain and spinal cord were unremarkable. Histologic examination revealed scattered subpial foci of demyelination throughout the brain stem, with involvement of the hippocampal formation bilaterally. Although occasional active lesions at early stages of development were noted, most lesions were gliotic and therefore quiescent. This case and one similar example of disseminated subpial demyelination found in the literature probably represent an unusual variant of multiple sclerosis.     

Multiple sclerosis and non-communicating syringomyelia: a casual association or linked diseases?

Non-communicating syringomyelia (NCS) can be associated with different disease processes such as arachnoidytis, trauma or tumor. Approximately 12 cases, documented radiographically, of the association of non-communicating syringomyelia and multiple sclerosis (MS) have been described but their relationship remains obscure. In 3 patients with laboratory supported MS spinal magnetic resonance imaging (MRI) revealed a central cystic cavity. In 2 patients lesions on the spinal cord above the cavity were demonstrated. At 3-year follow-up in 1 patient, no change in the cavity was detected. Although 3 cases are insufficient for providing a definitive conclusion on the relationship between these two diseases, we suggest that demyelinating lesions have to be regarded as possible causes of spinal, asymptomatic cavities.     

Widespread maculopapular rash due to intramuscular interferon beta-1a during the treatment of multiple sclerosis

We describe a 41-year-old woman with multiple sclerosis, who presented erythematous maculopapular rash on the trunk and extremities after the second injection of interferon beta-1a. Histopathologic examination of the lesion revealed lymphocytic exocytosis and perivascular lymphocytic infiltrate in the dermis. Oral antihistamine and topical corticosteroid was started. After improvement of the lesions, the third injection was performed. However, the same reaction occurred. A prick test, which was performed 6 weeks after the eruption, also revealed positive reaction. Although injection-site reactions have been observed with interferon beta-1a, to our knowledge there have been no previous reports of interferon beta-1a-induced widespread cutaneous reaction.     

Multiple brain metastases from malignant thymoma.

A rare case of thymic carcinoma with multiple brain metastasis is reported. In our extensive review of the literature only six of 30 reports of intracranial thymoma metastasis describe multiple metastases. A 38-year-old man presented with signs of raised intracranial pressure that had began 15 days previously. Cranial MRI revealed over 70 cystic lesions in the supra and infratentorial regions. Stereotactic biopsy was planned. On the second day of his admission he deteriorated and died the following day. The autopsy revealed a mass in the mediastinum. In the brain parenchyma were multiple cystic lesions between 0.5 and 3 cm in diameter. Histopathologically they were diagnosed as metastases from the thymic carcinoma. The mean survival with a single brain metastasis is approximately 256 days, whereas with multiple brain metastases it is only 64 days, thus treatment of this tumor demands prompt surgery whenever possible and optimal adjuvant therapy.     

Detection of novel rearrangement of the JC virus gene in a case of progressive multifocal leukoencephalopathy with adult T-cell leukemia]

A 53-year-old man with adult T-cell leukemia (ATL) developed progressive left hemiparesis and left homonymous hemianopsia. Magnetic resonance imaging (MRI) one month later showed multiple high-intensity lesions in the white matter of both occipital lobes, with predominance in the right side. Detection of JCV genome with polymerase chain reaction in his cerebrospinal fluid subsequently confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML). He was admitted to our hospital. The serum level of soluble interleukin-2 receptor in the patient increased, and both edema and new Gd-enhanced lesions were observed in the cortex of the occipital lobe. He was treated with systemic administrations of Pirarubicin. Cyclophosphamide, and Prednisolone. as well as intrathecal injection of Methotrexate and Cytarabine. Although these treatments temporarily alleviated the symptoms of PML. the ATL spread to the liver and kidney. He died of multiple organ failure. Analysis of his JCV genes revealed that there were three types of rearrangements in the regulatory domains of the JCV genes. All three types lacked the domain B. and two had duplicate domain A. This is the first report of the simultaneous detection of three different types of rearrangements in JCV genes in a single patient. It has been reported that white-matter lesions caused by typical PML are not enhanced in Gd-MRI. However. the lesions seen in this patient were enhanced in Gd-MRI. Such enhancement might be attributable to the modification of the lesions through the direct invasion of ATL cells to the central nervous system.     

Multiple cerebral and leptomeningeal metastases from ovarian carcinoma: unusual early presentation.

Although virtually any systemic malignancy is capable of metastasizing to the brain, ovarian carcinoma, one of the more common female genital malignancies, is one of the rarer forms of brain metastases. In general, the outcome for ovarian carcinoma with brain metastases is extremely poor as most of these patients have widespread lesions elsewhere. This report describes the first known case of multiple cerebral and leptomeningeal metastases as the initial manifestation of ovarian carcinoma in a 41-year old woman who presented with a one-week history of headache, vomiting and confusion. CT scan of the brain was unremarkable, but lumbar puncture revealed atypical cells in the CSF. MRI scan of the brain showed multiple small enhancing lesions. Craniotomy for excision of one of these lesions demonstrated metastatic adenocarcinoma. A large ovarian tumour identified on pelvic CT scan was resected and the patient subsequently received chemotherapy and radiotherapy. Unfortunately she continued to decline and died within six months. Unlike primary tumours such as malignant melanoma, ovarian carcinoma does not have a predilection for the central nervous system (CNS), but the rare instances with CNS involvement occur at an advanced stage of the disease. Once the CNS is involved, the outcome is abysmal, even with multimodality therapy. It is extremely unusual for ovarian carcinoma to present with multiple CNS involvement.     

A case of central pontine and extra-pontine myelinolysis demonstrated by magnetic resonance imaging]

A 74-year-old woman was admitted because of disturbed consciousness due to iatrogenic hyponatremia. At admission, her serum level of sodium was 88 mEq/l. The hyponatremia was carefully corrected with physiologic saline for three days at a rate of 0.75 mEq/h until the serum sodium concentration of 135 mEq/l was achieved. Her neurologic state gradually improved to respond to painful stimuli with her left hand during the first week of treatment, but further improvement was not noted after that. On the 16th hospital day, seizure developed on her face and left upper extremity. Although abnormal lesions were not detected by MRI of the brain at admission, those performed on the 15th hospital day revealed multiple uncommon lesions with Tl and Gd enhanced images. On Tl images areas of low signal intensity were found in the central pons and left putamen, which were more clearly demonstrated by Gd enhanced MRI images. These lesions were considered to be consistent with central pontine and extra-pontine myelinolysis (CPEM). In addition, a part of the right temporal cortex was enhanced clearly by Gd-DTPA, which was also thought to be an extra-pontine myelinolysis. These lesions were responsible for the development of neurological signs due to CPEM. To our knowledge, this is the first case in which a lesion in the cerebral cortex was revealed as an extra-pontine myelinolysis by Gd enhanced MRI images. Gd enhanced MRI is useful for detecting the extra-pontine myelinolysis as well as central pontine myelinolysis.     

Parkinsonism and multiple sclerosis-Is there association?

Association between multiple sclerosis (MS) and parkinsonism is rarely reported. We describe clinical, radiological and DAT scan findings in two patients presenting with parkinsonism. MRI revealed demyelinating lesions of the central nervous system consistent with MS in both patients. On the other hand, DAT scan findings were supportive of Parkinson's disease. There is still an open debate whether MS lesions can cause parkinsonism, or these are just coincidental findings of two different diseases in the same patient. Although there are cases of causal relationship between parkinsonism and MS, some literature reports and our observations suggest that Parkinson's disease and MS can coexist as two separate diseases in the same patient. It is possible that the symptoms of Parkinson's disease can be aggravated by MS plaques, explaining the favorable response to corticosteroids in some patients.     

Sequential magnetic resonance imaging findings in hypereosinophilia-induced encephalopathy

Hypereosiophilia-induced encephalopathy (HE) is a rare but well-described clinical syndrome. However, serial magnetic resonance imaging (MRI) findings of HE have rarely been reported. We describe serial MRI findings of three patients with HE. The patients presented with acute confusion, focal neurological deficits and/or seizures. Eosinophils in repeated blood tests were more than 3000/mm³ in all the patients. Echocardiography in two patients showed findings consistent with eosinophilic endomyocardial fibrosis or global hypokinesia. The initial MRI revealed multiple high-signal lesions on T2-weighted images with gadolinium-DTPA enhancement on T1-weighted images, which were predominantly distributed in the border zone of the middle-anterior cerebral arteries and the middle-posterior cerebral arteries. The second MRIs taken prior to the initiation of steroid therapy showed that the lesions increased in size and number in the same area. The third MRIs performed long after the therapy showed that the lesions were shrunken. A brain biopsy specimen in one patient showed reactive gliosis following infarction with abundant intravascular eosinophils. The MRI-identified lesions in the patients with HE thus develop mainly in the border zone. The lesions occasionally increase in size and number and shrink if the eosinophilia is adequately treated. Although the nature of the MRI-identified lesions remains unclear, their pathogenesis may be related to multiple embolisms associated with concomitant cardiac abnormality and hypercoagulable state. Received: 18 July 2000, Received in revised form: 26 September 2000, Accepted: 24 October 2000     

Multiple myeloma presenting with multiple subcutaneous masses]

A 92-year-old female was admitted to our hospital with 2-months history of rapidly enlarging subcutaneous masses in the multiple skull's regions. Neurological examination in the admission showed slightly right hemiparesis. Skull X-P showed multiple osteolytic lesions, and CT showed high density masses. MRI revealed iso-intensity masses on both T1 and T2-weighted images. Gd-DTPA enhanced T1-weighted image showed masses with marked homogeneous enhancement like the dural tail sign in the dura adjacent to the tumors. The tumor in the right frontal subcutaneous region was partialy removed; this mass was diagnosed as multiple myeloma. Because the prognosis of such a case was very poor and she was older, we thought her quality of life (QOL) and she was conservatively treated. A case of multiple myeloma having plasmacytoma in multiple skull's regions was reported. Although 30 cases in the literature of multiple myeloma forming cranial or intracranial plasmacytoma were briefly reviewed, multiple lesion was not reported and we thought it as a very rare case. And if such a case was performed to remove all masses, we believed that three dimensional computed tomography images to distinguish the tumors from skull and skin (Perspective 3D-CT; SIEMES's Somatom Emotion 6 and Syngo) was very valuable for the preoperative evaluation of a surgical approach.