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2.
Rev Neurol (Paris) ; 162(3): 363-70, 2006 Mar.
Article in French | MEDLINE | ID: mdl-16585892

ABSTRACT

INTRODUCTION: Efficacy of high frequency subthalamic nucleus (STN) stimulation has been demonstrated in idiopathic Parkinson's disease (IPD). However, since it may be difficult to differentiate IPD from multiple system atrophy with parkinsonian presentation (MSA-P), a few cases of MSA-P has been treated by deep brain stimulation (DBS) and showed no sustained improvement of clinical signs. We report a patient with a clinical misdiagnosed MSA-P, later confirmed by neuropathological study, who was improved by DBS for one year. CASE REPORT: A 63-year-old parkinsonian patient had been treated by levodopa for 6 years with a persistent good response. Over one year he progressively developed disabling fluctuations with severe axial syndrome and vegetative non motor symptoms in off periods. After checking usual contraindications, he was included in surgical procedure protocol (bilateral STN stimulation). During the first year after surgery, the clinical status improved with disappearance of non motor fluctuations, a 45 percent decrease of the OFF UPDRS III score, and a 39 percent reduction of the treatment. However after one year, axial symptoms reappeared with recurrent falls, as well as increasing dysarthry and swallowing difficulties which were only slightly improved by levodopa. He developed severe urinary disorders increased by a prostatic adenoma which led to surgical treatment. During the post operative period, 2 years after DBS, he died suddenly from an unexplained cause. A cerebral autopsy was performed and showed a good position of the two electrodes in the STN. Microscopic studies revealed severe neuronal depletion in the substantia nigra but no Lewy bodies. Immunohistochemical methods demonstrated numerous argyrophilic glial cytoplasmic inclusions positive for alpha-synuclein and ubiquitin in the STN, putamen, globus pallidus, pontine nuclei and cerebellar white matter, significant of MSA. CONCLUSION: This case shows that DBS can improve parkinsonian signs in MSA-P with persistent dopa sensitivity. However, probably because of striatal degeneration progression, this improvement is time limited and STN DBS cannot be recommended in MSA.


Subject(s)
Deep Brain Stimulation , Multiple System Atrophy/therapy , Subthalamic Nucleus , Adenoma/surgery , Antiparkinson Agents/therapeutic use , Biomarkers , Brain/pathology , Brain Chemistry , Combined Modality Therapy , Diagnostic Errors , Disease Progression , Fatal Outcome , Humans , Levodopa/therapeutic use , Magnetic Resonance Imaging , Male , Middle Aged , Multiple System Atrophy/diagnosis , Multiple System Atrophy/metabolism , Multiple System Atrophy/pathology , Parkinson Disease/diagnosis , Parkinson Disease/drug therapy , Parkinson Disease/therapy , Prostatectomy , Prostatic Neoplasms/surgery , Substantia Nigra/pathology , Subthalamic Nucleus/physiopathology , Ubiquitin/analysis , alpha-Synuclein/analysis
3.
Rev Neurol (Paris) ; 161(11): 1079-90, 2005 Nov.
Article in French | MEDLINE | ID: mdl-16288173

ABSTRACT

INTRODUCTION: Vogt-Koyanagi-Harada syndrome is a rare, inflammatory disease with manifestations affecting the ocular, central nervous, audito-vestibular, and integumentary systems. Vogt-Koyanagi-Harada syndrome is more frequent in Asia but is also described in Europe. We report three new non asiatic cases of this syndrome. OBSERVATIONS: The three patients had bilateral panuveitis and hypoacusia. Two of them had peripheral facial palsy, two of them had vestibular syndrome and one of them lymphocytic meningitis. In each case we found characteristic HLA II typing and in one case we discovered the simultaneous presence of three auto-antibodies: anti-retina (anti-Arrestin type), anti-choroid and anti-cochlea. These patients were treated by corticosteroids but required an additional treatment by cyclophosphamide (0.8g/m2). The clinical course was favorable with visual sequelae for two and auditory one for one. DISCUSSION: These biological and therapeutic elements and a review of the recent literature are in favor of an autoimmune origin of this syndrome.


Subject(s)
Uveomeningoencephalitic Syndrome/physiopathology , Adult , Anti-Inflammatory Agents/therapeutic use , Autoantibodies/immunology , Choroid/immunology , Cochlea/immunology , Cyclophosphamide/therapeutic use , Drug Administration Schedule , Facial Paralysis/complications , Facial Paralysis/diagnosis , Facial Paralysis/physiopathology , Female , Hearing Loss/complications , Hearing Loss/diagnosis , Hearing Loss/physiopathology , Humans , Male , Middle Aged , Panuveitis/complications , Panuveitis/diagnosis , Panuveitis/physiopathology , Retina/immunology , Uveomeningoencephalitic Syndrome/drug therapy , Uveomeningoencephalitic Syndrome/immunology , Vestibular Diseases/complications , Vestibular Diseases/diagnosis , Vestibular Diseases/physiopathology
4.
Rev Neurol (Paris) ; 160(12): 1203-6, 2004 Dec.
Article in French | MEDLINE | ID: mdl-15602369

ABSTRACT

INTRODUCTION: Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant peripheral neuropathy characterized by compressive focal neuropathies and an underlying sensorimotor demyelinative polyneuropathy. It is usually caused by a 1.5 Mb deletion of the PMP22 gene (17p11.2). CASE REPORT: We describe the case of a 31 year-old woman who presented with acute demyelinative peripheral polyneuropathy affecting the four limbs and elevated cerebrospinal fluid protein content a few days after a viral illness. Acute inflammatory demyelinating polyneuropathy (AIDP, Guillain-Barré syndrome) was suspected. However, electrophysiologic examination suggested HNPP and subsequent genetic testing was confirmatory. CONCLUSION: This case demonstrates that HNPP can present in an acute manner, mimicking AIDP.


Subject(s)
Guillain-Barre Syndrome/etiology , Heredodegenerative Disorders, Nervous System/complications , Polyneuropathies , Acute Disease , Adult , Female , Heredodegenerative Disorders, Nervous System/diagnosis , Humans , Paralysis/complications , Pressure
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