Clinical Neuroscience

[UNCOMMON MANIFESTATION OF CENTRAL NERVOUS SYSTEM SARCOIDOSIS]

RÓZSA Anikó, SZTANKANINECZ Yvette, GÁCS Gyula, MAGYAR Tamás

JANUARY 20, 2007

Clinical Neuroscience - 2007;60(01-02)

[Two cases of uncommon manifestation of central nervous system sarcoidosis are reported. A 42 year-old man had a spinal cord sarcoidosis. MRI of the spinal cord showed myelopathy in the cervico-thoracic region, and the T2-weighted image showed increasing signal intensity. Neurological symptoms did not correllate with radiological abnormalities. Neurological manifestation was paucisymptomatic. Half a year later steroid and azatioprin therapy led to almost complet radiological and clinical regression. In the second case we present a 49 year-old woman who had left side internuclear ophthalmoplegia and the brainstem lesion. The patient was proven to have sarcoidosis. In this case no abnormalities were found in brain MRI. Neurological symptoms could not be detected by MRI, probably caused by brainstem parenchymal lesions consisting of microgranulomatosis that is sarcoid "brainstem encephalitis". Neurological symptoms improved after steroid treatment in this case too. In both of the cases pulmonary lymphadenopathy helped to diagnose sarcoidosis. In our cases there were interesting correllations between neurological symptoms and MRI abnormalities. At the spinal cord sarcoidosis the radiological abnormalities were more striking than the clinical manifestation. In the other case we found distinct brainstem symptoms but could not detect MRI abnormalities.]

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Clinical Neuroscience

[APPLICATION OF FUNCTIONAL MR-IMAGES ACQUIRED AT LOW FIELD IN PLANNING OF NEUROSURGICAL OPERATION CLOSE TO AN ELOQUENT BRAIN AREA]

AUER Tibor, SCHWARCZ Attila, JANSZKY József, HORVÁTH Zsolt, KOSZTOLÁNYI Péter, DÓCZI Tamás

[Aim of the study - Presentation of functional MRI performed at low magnetic field (1 Tesla) for planning microsurgical operation in a patient suffering from tumor close to an eloquent brain area. Methods - Microsurgical removal navigated by frameless stereotaxy of an intrinsic tumor located in eloquent area is indicated if speech function is not damaged, i.e. exact localisation and relationship of the tumor and speech area can be defined. Before operation an optimized EPI based 2D sequence was applied to yield functional MR images. At the planning of the operation the paradigm used for the localization of the sensory language cortex contained passive listening to a text. Control investigations were performed one month postoperatively. A specific psychological test, as an additional investigation to estimate the accurate level of the sensory language function, was also conducted. Results - Low resolution (matrix of 64×64) functional MR images visualized sensory speech center and auditory cortex satisfactorily. The scans showed clearly that the Wernicke's region was situated just above the tumor (WHO grade II glioma), and this finding increased the safety of intraoperative localization and reduced the risk of morbidity. Control examinations revealed minimal decrease in sensory language function, however, it was not noticeable for either the patient or her surroundings. Conclusion - Optimized functional MR imaging performed at low magnetic field can support planning of neurosurgical operations and reduce the morbidity of microsurgical interventions.]

Clinical Neuroscience

[CLINICAL EXPERIENCE WITH LEVETIRACETAM FOR ADULTS WITH EPILEPSY]

BARCS Gábor, SZŰCS Anna

[Objective - A retrospective study to evaluate the efficacy of levetiracetam in the treatment of adult pharmacoresistant epilepsy. Method - Retrospective work up of our treatmentexperiences with 55 pharmacoresistant patients treated with levetiracetam (11 of them on monotherapy) for 6-39 months. Three treatment groups were analysed: idiopathic generalised epilepsy (9 patients); partial epilepsy (30 patients); malignant or malignated epileptic syndromes (16 patients). Result - Seven idiopathic generalised patients (77%) and 5 partial epilepsy patients (16%) became seizure free. One idiopathic generalised epileptic patient, 10 partial epilepsy patients (33%) significantly improved. Six patients (37%) from the group of malignant or malignated epileptic syndromes also significantly improved. Five of the improved idiopathic generalised epilepsy patients and 6 of the improved partial epilepsy patients received levetiracetam monotherapy. Altogether seven patients (12% of the whole population) relapsed after a 4-15 months improved period. Fifteen patients (27%) suffered side effects (mainly somnolence, headache, dizziness and irritability) improving after dose reduction of levetiracetam (generally below 2000 mg pro day). Conclusion - Levetiracetam is an effective, well tolerable, broad-spectrum drug as adjunctive treatment or monotherapy in adult patients unsuccessfully treated with other antiepileptic drugs.]

Clinical Neuroscience

[NONSENSE MUTATION 193C>T OF NEUROFIBROMATOSIS TYPE 2 - A NEUROSURGICAL CHALLENGE]

BOBEST Mátyás, TÓTH Csaba, GYURCSÓ Mária, MOLNÁR Mária Judit, GARZULY Ferenc

[A 15 years old male was operated because of incidentally found intercostal schwannoma. Two years later severe cerebellar ataxy and left sided anacusis developed. MRI revealed bilateral vestibularis tumors and multiple cervical intradural extramedullar myelon compressing lesions. After partial resection of the huge left sided cerebello-pontin tumor, histologically schwannoma, and the exstirpation of the multiple cervical meningiomas the patient died three months later due to septic complications. The 24 years old mother had been operated on similar lesions 12 years earlier, after two weeks postoperative period she died. Her 14 years old twins are living, a boy also with bilateral acustic tumours and a girl who is intact. Genetic investigation revealed C>T nonsense mutation at position 193 in the exon 2 of the NF2 gene. This mutation cause premature truncation of the gene protein and is probably in connection with the clinically severe phenotype. Early diagnosis of this type of neurofibromatosis is mandatory concerning the therapy.]

Clinical Neuroscience

[UPDATES IN PRACTICAL NEUROLOGY - I. THE PRINCIPLES OF MODERN LEVODOPA THERAPY IN PARKINSON’S DISEASE]

KLIVÉNYI Péter, VÉCSEI László

[Despite the levodopa is used for the treatment of Parkinson’s disease for a long time, recently many questions raised about its clinical use. New issues emerged based on the clinical trials, on latest neuroimaging data and on better understanding the pathomechanism of motor complications. These observations have changed the routine clinical use of levodopa. In this review we summarize the evidences and practical implications of levodopa therapy.]

Clinical Neuroscience

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[The value of motor evoked potentials in the diagnosis of spondylotic myelopathy]

SIMÓ Magdolna, ARÁNYI Zsuzsanna

[Introduction - Motor evoked potential (MEP) is the only method that is able to assess the function of the corticospinal tract in various neurological conditions, such as myelopathies. Myelopathy associated with cervical spondylosis, especially at an early stage, has often slight and non-specific clinical signs, pointing to the importance of the electrophysiological assessment of the spinal cord. The authors' aim was to investigate the sensitivity of MEP examination in the detection of myelopathy secondary to cervical spondylosis. Patients and methods - Patients were classified into three groups according to clinical signs and symptoms: Group I includes patients who have cervical spondylosis as demonstrated by MRI (narrowing of the spinal canal, discal herniation, spinal cord compression) but no complaints or signs suggestive of myelopathy. Results - In Group II patients had minor, non-specific complaints, such as paraesthesia of the legs and gait disturbance raising the possibility of myelopathy, but neurological examination revealed no pyramidal signs. In Group III patients had pyramidal signs as well. In Group I corticospinal function was normal in all patients, as assessed by MEP examination. In Group II all patients had prolonged central motor conduction time or absent responses to cortical stimulation. Likewise, in Group III MEP revealed abnormal corticospinal function in all patients but one. Conclusions - In summary, MEP proved sensitive in the detection of corticospinal dysfunction in myelopathy associated with cervical spondylosis at a stage when clinical signs of pyramidal lesion are not yet present and patients have only minor complaints. On the other hand, if patients are completely symptom free with regard to myelopathy, MEP is also unlikely to disclose corticospinal dysfunction. If pyramidal lesion is evident already by clinical examination, MEP provides no further help. ’Falsenegative’ results are also possible.]

Clinical Neuroscience

[Common variable immunodeficiency with coexisting central nervous system sarcoidosis. Case report and literature review with implications for diagnosis and pathogenesis]

MAGDALENA Dziadzio, HORTOBÁGYI Tibor, DESMOND Kidd, RONNIE Chee

[We describe a patient with a history of longstanding primary generalised epilepsy, on anticonvulsant therapy, who presented with fever, headache, worsening seizures and hallucinations. Among various investigations, the patient had high CSF protein and ACE levels, leptomeningeal nodular enhancement on MRI brain and non-caseating granulomas in the brain and meninges on the biopsy. The patient was diagnosed with neurosarcoidosis. Subsequently, he was found to be panhypogammaglobulinaemic and was diagnosed with probable common variable immunodeficiency (CVID). The coexistence of common variable immunodeficiency and neurosarcoidosis is rare. Typically, non-caseating granulomas in CVID patients are localised in the lymphatic tissue and solid organs. To our knowledge, there are only five reports of the granulomas of the central nervous system (CNS) in CVID. We discuss the diagnostic difficulties in this case and review the literature.]

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[In this study, we present two cases of different eye movement disorders with variable case histories but with the same end stage; abduction paresis of one of the eyes, which ceased when the other eye was covered. Our differential diagnosis is that either the ocular form of myasthenia gravis, convergence spasm or ocular myotonia could explain the symptoms. However, we hypothesize that the clinical picture corresponds to pseudo abducens palsy or focal dystonia of the extraocular muscle, which in turn could be the result of impaired inhibition of the tonic resting activity of the antagonistic medial rectus muscle. We offer an explanation for the patomechanism of pseudoabducens palsy and the variants of internuclear ophthalmoplegia.]