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PO17-TU-09 Pituitary apoplexy presenting with bilateral third nerve palsy and bilateral proptosis: case report
19th World Congress of Neurology, Poster Abstracts / Journal of the Neurological Sciences 285 S1 (2009) S155–S339
S241
Conclusion: In our case infliximab treatment was dramatic useful for a young man affected by NS resistant to corticosteroids and immunosuppressive therapy.
Conclusion: The results emphathized the importance of interferon-a treatment in modulating the neurological complications related to HCV infection.
PO17-TU-06 Superficial siderosis, a rare cause of progressive ataxia
PO17-TU-08 The multiple faces of Fahr calcifications
P. Lolekha, K. Kulkantrakorn. Neurology Division, Department of Internal Medicine, Thammasat University, Pathumthani, Thailand
I. Ben Hamouda1 , M.N. Tougourti2 , N. Khamassi2 , M. Hamza2 . 1 Neurology, Charles Nicolle Hospital, Tunis, Tunisia; 2 Medicine, Razi Hospital-La Manouba, Manouba, Tunisia
Purpose: To report a case of patient who presented with progressive gait ataxia caused by superficial siderosis. Method: A 51-year-old male patient presented with an eleven year history of progressive gait unsteadiness. He had to walk with support during the first 4 years of illness without weakness or sensory symptoms. His wife also noticed that his hearing was impaired and he was advised by his doctor to use hearing aids. Four years later, he had to use wheelchair due to severe gait ataxia and he became almost deaf. He had never experienced any episode of headache, nausea, vomiting or vertigo. Family history was unremarkable for neurodegenerative diseases. General physical examination was unremarkable. Neurological examination revealed bilateral gaze-evoked nystagmus, sensorineural hearing loss in both ears, spastic tone of both legs. Deep tendon reflexes were increased in all extremities. Babinski’s sign was dorsiflexion response. Mild finger to nose test impairment and dysdiadokokinesia were detected on both sides. He could not perform tandem gait due to severe gait ataxia. Results: Laboratory tests which included CBC, BUN, creatinine, liver function test and coagulogram were normal. MRI of the brain and spinal cord revealed atrophy of the cerebellar vermis and hemispheres as well as leptomeningeal hemosiderin deposition along the brain stem, cerebellum, temporal cortex and spinal cord. Lumbar puncture revealed xanthrochromia in CSF. Cerebral and spinal angiogram revealed no abnormalities. The clinical diagnosis in this case was idiopathic superficial siderosis of the central nervous system (SSCN). Conclusion: The initial diagnostic evaluation of an ataxic patient should include an assessment for potentially acquired causes. Superficial siderosis of the central nervous system (SSCN) is a rare disorder that can cause progressive gait ataxia. An early diagnosis and prompt intervention are necessary to prevent severe deficits. PO17-TU-07 Theraputic effect of interferon alpha on extra hepatic complications of hepatitis C virus infection D.O.A. Elsalamawy. Neurology, Faculty of Medicine, Alexandria, Egypt Objective: Is to evaluate the therapeutic effect of interferon alpha (IFN-a) on the neurological complications such as peripheral neuropathy associated with hepatitis C virus infection HCV and essential mixed cryoglobulinemia EMC. Methods: This study was carried out on 20 patients suffering from peripheral neuropathy, associated with EMC related to hepatitis C virus infection. All patients were submitted to full history taking, thorough clinical and neurological examinations, lab investigations including, fasting blood sugar, HCV antibodies and polymerase chain reaction (PCR), cryoglobulin, SGOT, SGPT, urea, creatinine. Electrophysiological study in the form of nerve conduction before and 6 months after IFN-a treatment. Sural nerve biopsies were obtained from two selected cases after written informed consent and were submitted to histopathological examination using Haematoxylin and Eosin (H and E) stained sections. Results: After six months of interferon-a treatment clinical and electrophysiological tests showed significant improvement of all parameters as well as lab investigations showed HCV RNA was negative in 18 (90%) of cases but serum cryoglobulins remained positive in most of cases 19 (95%).
Purpose: Basal ganglia calcifications are the hallmark of two entities: Fahr syndrome, associated to calcium disorder, mainly hypoparathyroidism, and Fahr disease, a distinct hereditary autosomal dominant pathology, with normal calcium metabolism. We report three cases of Fahr calcifications, illustrating the heterogeneity of this entity. Observations: Observation 1: A 39 years man, cognitive and psychotic symptoms and tetanic crisis since age 14. Clinical, biological and neuroimaging investigations conclude to a primitive hypoparathyrodism, with Fahr calcifications. Observation 2: A 68 years woman, complex partial epilepsy since age 58 and a few months after a subtotal thryoidectomy. Investigations led to the diagnosis of iatrogenic hypoparathyroidism and Fahr calcifications. Observation 3: A 65 years man, pneumonia and confusion. History of Klinefelter syndrome (caryotype 47XXY) and asthma. Cerebral TDM reveals typical Fahr calcifications. Calcium metabolism is normal. No familial history of intra-cerebral calcifications. One sibling has a Klinefelter syndrome. Discussion: Fahr syndrome as described by Theodor Fahr (1930) is an anatomoclinical entity characterized by symmetrical basal ganglia calcifications, not related to arteriosclerosis and associated with various clinical manifestations and phosphocalcium abnormalities. Fahr disease pathogenesis is poorly understood, since calcium metabolism is normal. In our first case, early onset of symptoms and cognitive deficit are in favour of Fahr disease, but hypoparathyroidism is against this diagnosis. The second case is typical of Fahr syndrome. Poorly controlled epilepsy, related to a severe iatrogenic and misdiagnosed hypocalcaemia, must be emphasised. Although, cerebral calcifications have been reported in the setting of hypogonadism but were rarely of Fahr type. Conclusion: Our observations underscore the pathologic frame heterogeneity of Fahr calcifications and raise some questions about their pathogenesis and origin. PO17-TU-09 Pituitary apoplexy presenting with bilateral third nerve palsy and bilateral proptosis: case report G. Ayberk1 , H.F. Komurcu2 , M.F. Ozveren1 , N. Ozturk2 , O. Anlar2 . Neurosurgery Section, Ataturk Egitim ve Arastirma Hastanesi, Ankara, Turkey; 2 Neurology Section, Ataturk Egitim ve Arastirma Hastanesi, Ankara, Turkey
1
Introduction: Pituitary apoplexy is a sudden neurologic impairment, usually due to a vascular process. It is characterized by a sudden onset of headache, visual symptoms, altered mental status, and hormonal dysfunction due to acute hemorrhage or infarction of a pituitary gland. Here we report a rare case with pituitary apoplexy presenting with bilateral proptosis and bilateral third nerve palsy. Case: Forty-five year-old man was operated due to coronary arter disease. On the first day of the cardiac operation, proptosis developed bilaterally. Neurologic examination revealed bilateral proptosis, bilateral third nerve palsy and restriction to the right lateral gaze in the left eye. MRI revealed macro adenoma with extension into the sphenoid sinus and cavernous sinus involvement bilaterally (Grade IV-C according to modified Hardy classification). The adenoma was hormonally inactive and the patient was operated by transnasal trans-sphenoidal route. Pathologic examination
S242
19th World Congress of Neurology, Poster Abstracts / Journal of the Neurological Sciences 285 S1 (2009) S155–S339
reported as null cell adenoma. The third nerve palsy improved partially at the first postoperative day. Discussion: Since the pituitary adenomas slowly growing tumors, and there is no dural barrier at the internal side of the diaphragm sella, the first structure compressed is the chiasm. Although the sixth nerve more adjacent to the pituitary gland, the third nerve is the most involved cranial nerve in pituitary tumor. An ocular nerve palsy resulting from encroachment of the cavernous sinus is less common but has been reported in 1–6% of patients with pituitary tumor, and generally develops as the end stage of a pituitary tumor. Ocular motor nerve involvement due to pituitary tumors is generally resulting from an apoplectic event in the literature. Conclusion: When unexpected bilateral third nerve palsy observed after any operation pituitary apoplexy should be suspected. PO17-TU-10 A case of laughter-induced syncope H. El Otmani1 , F. Moutaouakil1 , H. Fadel1 , M.A. Rafai2 , B. El Moutawakil2 , I. Slassi2 . 1 Neurology, Al Kortobi hospital, Tangier, Morocco; 2 Neurology, Ibn Rochd universitary hospital, Casablanca, Morocco Introduction: Laughter-induced syncope (or gelastic syncope) is a rare situation and likely goes unrecognized by many health care providers. Case report: A 65 year old men with no personal history of illness particularly diabetes or heart disease, was admitted to hospital because of 4 episodes of recurrent events of loss of consciousness exclusively induced by Giggling. The first episode occurred 8 months ago after listening to a joke. There were no other symptoms and physical examination, particularly neurological and cardiac was normal. All paraclinical investigations were also normal: Laboratory tests (glycaemia, thyroid function and blood cobalamin), cardiac and neurological investigations (electrocardiographic monitoring, echocardiography, electroencephalography and brain MRI). Treatment with propanolol led to a prevention of attacks. Conclusion: Sustained laughter is accompanied by repetitive bursts of forced expiration, corresponding to short repetitive Valsalva maneuvers. Laughter-induced syncope is thought to be a subtype of the vagally mediated syncopal attacks. Others differential diagnoses must be ruled out especially gelastic atonic seizures and cataplexy. Propanolol seems to be a good treatment to prevent recurrences. PO17-TU-11 Posterior leukoencephalopathy syndrome – case report S. Miljkovic, M. Arbutina, D. Racic, Z. Vujkovic. Neurology, Clinical Center Banjaluka, Banjaluka, Bosnia and Herzegovina Purpose: Posterior leukoencephalopathy syndrome is a rare neurological disorder which is characterised by white matter oedema affecting the occipital and posterior parietal lobs of the brain. Predisposing factors are eclampsia, renal failure, hypertension, some medicines. Clinical feature are seizures, visual aura, cortical blindness, confusion, headache, rarely focal deficit. Case: Our patient was PD, 19 years old woman who has just given birth. She was in stupor, agitated periodicaly. Before she admited she was complained to severe headache, and after that she had had seizure and somnolence to stupor. On the neurological examination we found no focal deficit, somnolentio, Babinski sign was positive bilateral. We also found hypertension RR 210/140. We made CT scan on admition and it was unspecific. Second day we made MRI and found patological IS on T2W/flair sequences in both cerebral and cereberal hemispheres (white matter lession). After that we assumed that it might be PRESS sy, and we start with therapy (aggressive antihypertensive therapy, antiedematose therapy, sedative). Third day after therapy patient were much better, with adequate concious, and without neurological deficit. After 7
days we made control MRI and found complete regression of earlier white matter lessions. Conclusions: Konwledge of posterior leukoencephalopathy syndrome is of great significance in everyday clinical practice. For diagnosis we need presence of clinical signs (headache, seizure, altered mental status . . . ) and MRI abnormalities (White matter lession). Clinician must be aware of this syndrome, and this syndrome is reversible by prompt lowering of raised blood pressure, or by stopping the administration of some medicines. PO17-TU-12 A Tunisian family with succinate dehydrogenase deficiency presenting with leucodystrophy associated to polydactyly I. Kraoua1 , S. Hamdi1 , H. Benrhouma1 , N. Fradj1 , A. Rouissi1 , K. Tlili2 , N. Gouider-Khouja1 . 1 Departement of Child and Adolescent Neurology, National Institute of Neurology, Tunis, Tunisia; 2 Departement of Radiology, Sahloul Hospital, Sousse, Tunisia Introduction: Succinate dehydrogenase deficiency (SDHD) is a very rare condition. SDH is part of both the Krebs cycle and the respiratory chain. SDHD often presents as others defects of respiratory chain with Kearns-Sayre syndrome, myopathy, cardiomyopathy or optic atrophy, ataxia. It has been rarely reported with leucoencephalopathy and or Leigh syndrome. Objective: To report a patient with SDHD and stress on the rare presentation as leucoencephalopathy with Leigh syndrome and unusual association to polydactyly. Case report: A 3 year-old-girl, second child of first degree relatives with family history of hexadactyly and deaths at early age, presented a progressive psychomotor regression and irritability at age 18 months. Examination showed dysmorphic features with polydactyly, axial hypotonia, spastic tetraparesis and trunk and upper limbs dystonia. Fundoscopic examination was normal. Electromyogram was normal. Screening for inborn errors of metabolism showed high blood and CSF lactate. Brain MRI showed an extensive leukoencephalopathy with involvement of periventricular regions, thalami and brainstem. MR Spectroscopy showed a pic of succinate and lactate suggesting the diagnosis of SDHD. Comments and conclusion: Our observation is remarkable because of the uncommon presentation as leukodystrophy with Leigh syndrome revealing SDHD and the association with polydactyly which has not been previously reported. The patient is undergoing enzymatic study to confirm diagnosis and genetic study for association SDHD and polydactyly to search for possible microrearrangements that could support the hypothesis of a “contiguous gene” syndrome. PO17-TU-13 Possession by malign spirits? An organic explanation for bizarre behaviour in 2 young women M.S. Chong, L. Nashef, A. Siddiqui, R.S. Delamont. Neurology, Kings College Hospital, London, United Kingdom Purpose: A new explanation for an old societal problem. Methods: We reviewed two recent clinical cases with similar presentations. Results: Case 1: A healthy 16 year old oriental woman was hospitalised with a four day history of confusion and abnormal behaviour. She became increasingly agitated, paranoid and combative Her father was convinced that she was “possessed” by “malign spirits” and insisted that she be treated by a “spiritual healer”. Respiratory failure needing ventilatory ITU support prevented this. After extensive investigations, anti-NMDA receptor antibodies were identified. She was treated and made a partial recovery with persisting mild memory and cognitive difficulties. Case 2: A healthy 25 year old Caucasian woman was admitted with tearful confusion nausea and anorexia. She was initially
S241
Conclusion: In our case infliximab treatment was dramatic useful for a young man affected by NS resistant to corticosteroids and immunosuppressive therapy.
Conclusion: The results emphathized the importance of interferon-a treatment in modulating the neurological complications related to HCV infection.
PO17-TU-06 Superficial siderosis, a rare cause of progressive ataxia
PO17-TU-08 The multiple faces of Fahr calcifications
P. Lolekha, K. Kulkantrakorn. Neurology Division, Department of Internal Medicine, Thammasat University, Pathumthani, Thailand
I. Ben Hamouda1 , M.N. Tougourti2 , N. Khamassi2 , M. Hamza2 . 1 Neurology, Charles Nicolle Hospital, Tunis, Tunisia; 2 Medicine, Razi Hospital-La Manouba, Manouba, Tunisia
Purpose: To report a case of patient who presented with progressive gait ataxia caused by superficial siderosis. Method: A 51-year-old male patient presented with an eleven year history of progressive gait unsteadiness. He had to walk with support during the first 4 years of illness without weakness or sensory symptoms. His wife also noticed that his hearing was impaired and he was advised by his doctor to use hearing aids. Four years later, he had to use wheelchair due to severe gait ataxia and he became almost deaf. He had never experienced any episode of headache, nausea, vomiting or vertigo. Family history was unremarkable for neurodegenerative diseases. General physical examination was unremarkable. Neurological examination revealed bilateral gaze-evoked nystagmus, sensorineural hearing loss in both ears, spastic tone of both legs. Deep tendon reflexes were increased in all extremities. Babinski’s sign was dorsiflexion response. Mild finger to nose test impairment and dysdiadokokinesia were detected on both sides. He could not perform tandem gait due to severe gait ataxia. Results: Laboratory tests which included CBC, BUN, creatinine, liver function test and coagulogram were normal. MRI of the brain and spinal cord revealed atrophy of the cerebellar vermis and hemispheres as well as leptomeningeal hemosiderin deposition along the brain stem, cerebellum, temporal cortex and spinal cord. Lumbar puncture revealed xanthrochromia in CSF. Cerebral and spinal angiogram revealed no abnormalities. The clinical diagnosis in this case was idiopathic superficial siderosis of the central nervous system (SSCN). Conclusion: The initial diagnostic evaluation of an ataxic patient should include an assessment for potentially acquired causes. Superficial siderosis of the central nervous system (SSCN) is a rare disorder that can cause progressive gait ataxia. An early diagnosis and prompt intervention are necessary to prevent severe deficits. PO17-TU-07 Theraputic effect of interferon alpha on extra hepatic complications of hepatitis C virus infection D.O.A. Elsalamawy. Neurology, Faculty of Medicine, Alexandria, Egypt Objective: Is to evaluate the therapeutic effect of interferon alpha (IFN-a) on the neurological complications such as peripheral neuropathy associated with hepatitis C virus infection HCV and essential mixed cryoglobulinemia EMC. Methods: This study was carried out on 20 patients suffering from peripheral neuropathy, associated with EMC related to hepatitis C virus infection. All patients were submitted to full history taking, thorough clinical and neurological examinations, lab investigations including, fasting blood sugar, HCV antibodies and polymerase chain reaction (PCR), cryoglobulin, SGOT, SGPT, urea, creatinine. Electrophysiological study in the form of nerve conduction before and 6 months after IFN-a treatment. Sural nerve biopsies were obtained from two selected cases after written informed consent and were submitted to histopathological examination using Haematoxylin and Eosin (H and E) stained sections. Results: After six months of interferon-a treatment clinical and electrophysiological tests showed significant improvement of all parameters as well as lab investigations showed HCV RNA was negative in 18 (90%) of cases but serum cryoglobulins remained positive in most of cases 19 (95%).
Purpose: Basal ganglia calcifications are the hallmark of two entities: Fahr syndrome, associated to calcium disorder, mainly hypoparathyroidism, and Fahr disease, a distinct hereditary autosomal dominant pathology, with normal calcium metabolism. We report three cases of Fahr calcifications, illustrating the heterogeneity of this entity. Observations: Observation 1: A 39 years man, cognitive and psychotic symptoms and tetanic crisis since age 14. Clinical, biological and neuroimaging investigations conclude to a primitive hypoparathyrodism, with Fahr calcifications. Observation 2: A 68 years woman, complex partial epilepsy since age 58 and a few months after a subtotal thryoidectomy. Investigations led to the diagnosis of iatrogenic hypoparathyroidism and Fahr calcifications. Observation 3: A 65 years man, pneumonia and confusion. History of Klinefelter syndrome (caryotype 47XXY) and asthma. Cerebral TDM reveals typical Fahr calcifications. Calcium metabolism is normal. No familial history of intra-cerebral calcifications. One sibling has a Klinefelter syndrome. Discussion: Fahr syndrome as described by Theodor Fahr (1930) is an anatomoclinical entity characterized by symmetrical basal ganglia calcifications, not related to arteriosclerosis and associated with various clinical manifestations and phosphocalcium abnormalities. Fahr disease pathogenesis is poorly understood, since calcium metabolism is normal. In our first case, early onset of symptoms and cognitive deficit are in favour of Fahr disease, but hypoparathyroidism is against this diagnosis. The second case is typical of Fahr syndrome. Poorly controlled epilepsy, related to a severe iatrogenic and misdiagnosed hypocalcaemia, must be emphasised. Although, cerebral calcifications have been reported in the setting of hypogonadism but were rarely of Fahr type. Conclusion: Our observations underscore the pathologic frame heterogeneity of Fahr calcifications and raise some questions about their pathogenesis and origin. PO17-TU-09 Pituitary apoplexy presenting with bilateral third nerve palsy and bilateral proptosis: case report G. Ayberk1 , H.F. Komurcu2 , M.F. Ozveren1 , N. Ozturk2 , O. Anlar2 . Neurosurgery Section, Ataturk Egitim ve Arastirma Hastanesi, Ankara, Turkey; 2 Neurology Section, Ataturk Egitim ve Arastirma Hastanesi, Ankara, Turkey
1
Introduction: Pituitary apoplexy is a sudden neurologic impairment, usually due to a vascular process. It is characterized by a sudden onset of headache, visual symptoms, altered mental status, and hormonal dysfunction due to acute hemorrhage or infarction of a pituitary gland. Here we report a rare case with pituitary apoplexy presenting with bilateral proptosis and bilateral third nerve palsy. Case: Forty-five year-old man was operated due to coronary arter disease. On the first day of the cardiac operation, proptosis developed bilaterally. Neurologic examination revealed bilateral proptosis, bilateral third nerve palsy and restriction to the right lateral gaze in the left eye. MRI revealed macro adenoma with extension into the sphenoid sinus and cavernous sinus involvement bilaterally (Grade IV-C according to modified Hardy classification). The adenoma was hormonally inactive and the patient was operated by transnasal trans-sphenoidal route. Pathologic examination
S242
19th World Congress of Neurology, Poster Abstracts / Journal of the Neurological Sciences 285 S1 (2009) S155–S339
reported as null cell adenoma. The third nerve palsy improved partially at the first postoperative day. Discussion: Since the pituitary adenomas slowly growing tumors, and there is no dural barrier at the internal side of the diaphragm sella, the first structure compressed is the chiasm. Although the sixth nerve more adjacent to the pituitary gland, the third nerve is the most involved cranial nerve in pituitary tumor. An ocular nerve palsy resulting from encroachment of the cavernous sinus is less common but has been reported in 1–6% of patients with pituitary tumor, and generally develops as the end stage of a pituitary tumor. Ocular motor nerve involvement due to pituitary tumors is generally resulting from an apoplectic event in the literature. Conclusion: When unexpected bilateral third nerve palsy observed after any operation pituitary apoplexy should be suspected. PO17-TU-10 A case of laughter-induced syncope H. El Otmani1 , F. Moutaouakil1 , H. Fadel1 , M.A. Rafai2 , B. El Moutawakil2 , I. Slassi2 . 1 Neurology, Al Kortobi hospital, Tangier, Morocco; 2 Neurology, Ibn Rochd universitary hospital, Casablanca, Morocco Introduction: Laughter-induced syncope (or gelastic syncope) is a rare situation and likely goes unrecognized by many health care providers. Case report: A 65 year old men with no personal history of illness particularly diabetes or heart disease, was admitted to hospital because of 4 episodes of recurrent events of loss of consciousness exclusively induced by Giggling. The first episode occurred 8 months ago after listening to a joke. There were no other symptoms and physical examination, particularly neurological and cardiac was normal. All paraclinical investigations were also normal: Laboratory tests (glycaemia, thyroid function and blood cobalamin), cardiac and neurological investigations (electrocardiographic monitoring, echocardiography, electroencephalography and brain MRI). Treatment with propanolol led to a prevention of attacks. Conclusion: Sustained laughter is accompanied by repetitive bursts of forced expiration, corresponding to short repetitive Valsalva maneuvers. Laughter-induced syncope is thought to be a subtype of the vagally mediated syncopal attacks. Others differential diagnoses must be ruled out especially gelastic atonic seizures and cataplexy. Propanolol seems to be a good treatment to prevent recurrences. PO17-TU-11 Posterior leukoencephalopathy syndrome – case report S. Miljkovic, M. Arbutina, D. Racic, Z. Vujkovic. Neurology, Clinical Center Banjaluka, Banjaluka, Bosnia and Herzegovina Purpose: Posterior leukoencephalopathy syndrome is a rare neurological disorder which is characterised by white matter oedema affecting the occipital and posterior parietal lobs of the brain. Predisposing factors are eclampsia, renal failure, hypertension, some medicines. Clinical feature are seizures, visual aura, cortical blindness, confusion, headache, rarely focal deficit. Case: Our patient was PD, 19 years old woman who has just given birth. She was in stupor, agitated periodicaly. Before she admited she was complained to severe headache, and after that she had had seizure and somnolence to stupor. On the neurological examination we found no focal deficit, somnolentio, Babinski sign was positive bilateral. We also found hypertension RR 210/140. We made CT scan on admition and it was unspecific. Second day we made MRI and found patological IS on T2W/flair sequences in both cerebral and cereberal hemispheres (white matter lession). After that we assumed that it might be PRESS sy, and we start with therapy (aggressive antihypertensive therapy, antiedematose therapy, sedative). Third day after therapy patient were much better, with adequate concious, and without neurological deficit. After 7
days we made control MRI and found complete regression of earlier white matter lessions. Conclusions: Konwledge of posterior leukoencephalopathy syndrome is of great significance in everyday clinical practice. For diagnosis we need presence of clinical signs (headache, seizure, altered mental status . . . ) and MRI abnormalities (White matter lession). Clinician must be aware of this syndrome, and this syndrome is reversible by prompt lowering of raised blood pressure, or by stopping the administration of some medicines. PO17-TU-12 A Tunisian family with succinate dehydrogenase deficiency presenting with leucodystrophy associated to polydactyly I. Kraoua1 , S. Hamdi1 , H. Benrhouma1 , N. Fradj1 , A. Rouissi1 , K. Tlili2 , N. Gouider-Khouja1 . 1 Departement of Child and Adolescent Neurology, National Institute of Neurology, Tunis, Tunisia; 2 Departement of Radiology, Sahloul Hospital, Sousse, Tunisia Introduction: Succinate dehydrogenase deficiency (SDHD) is a very rare condition. SDH is part of both the Krebs cycle and the respiratory chain. SDHD often presents as others defects of respiratory chain with Kearns-Sayre syndrome, myopathy, cardiomyopathy or optic atrophy, ataxia. It has been rarely reported with leucoencephalopathy and or Leigh syndrome. Objective: To report a patient with SDHD and stress on the rare presentation as leucoencephalopathy with Leigh syndrome and unusual association to polydactyly. Case report: A 3 year-old-girl, second child of first degree relatives with family history of hexadactyly and deaths at early age, presented a progressive psychomotor regression and irritability at age 18 months. Examination showed dysmorphic features with polydactyly, axial hypotonia, spastic tetraparesis and trunk and upper limbs dystonia. Fundoscopic examination was normal. Electromyogram was normal. Screening for inborn errors of metabolism showed high blood and CSF lactate. Brain MRI showed an extensive leukoencephalopathy with involvement of periventricular regions, thalami and brainstem. MR Spectroscopy showed a pic of succinate and lactate suggesting the diagnosis of SDHD. Comments and conclusion: Our observation is remarkable because of the uncommon presentation as leukodystrophy with Leigh syndrome revealing SDHD and the association with polydactyly which has not been previously reported. The patient is undergoing enzymatic study to confirm diagnosis and genetic study for association SDHD and polydactyly to search for possible microrearrangements that could support the hypothesis of a “contiguous gene” syndrome. PO17-TU-13 Possession by malign spirits? An organic explanation for bizarre behaviour in 2 young women M.S. Chong, L. Nashef, A. Siddiqui, R.S. Delamont. Neurology, Kings College Hospital, London, United Kingdom Purpose: A new explanation for an old societal problem. Methods: We reviewed two recent clinical cases with similar presentations. Results: Case 1: A healthy 16 year old oriental woman was hospitalised with a four day history of confusion and abnormal behaviour. She became increasingly agitated, paranoid and combative Her father was convinced that she was “possessed” by “malign spirits” and insisted that she be treated by a “spiritual healer”. Respiratory failure needing ventilatory ITU support prevented this. After extensive investigations, anti-NMDA receptor antibodies were identified. She was treated and made a partial recovery with persisting mild memory and cognitive difficulties. Case 2: A healthy 25 year old Caucasian woman was admitted with tearful confusion nausea and anorexia. She was initially