1
Poster Abstracts Wednesday, November 9, 2005 $319 striatal haematoma. The patient became well after adjunction of haloperidol treatment. The MRI showed the integrity of the corpus luysii ill both cases. Conclusion: Authors underline the rarity of hemiballismus due to all extraluysiall lesion and discuss its physiopathological, clinical, para- clinical and evolutionary aspects. 0859 Gilles De La Tourtte's Syndrome- A case series from India Anand, KS 1, Garg, S 2, Gurtoo, A 3. 2Dr. Ram Manhoar Lohia Hospital, Delhi, India; 2Maulana Azad Medical College, Lok Nayak Hospital, Delhi, India; 3Lady Hardinge Medical College, Smt. Sucheta Kriplani Hospital, Delhi, India Background: Gilles de la tourette"s Syndrome (GTS) is a neuro- psyclfiatric disorder characterized by motor and vocal tics associated with obsessive compulsive disorder (OCD) and attention deficit hyperactivity disorder (ADHD). Tiffs sylldrofme is often misdiagnosed as psychiatric disorder, therefore neuropsychiatric evaluation in patients suffering from it is very essential to make the correct diagnosis. Few reports, mostly single case reports of GTS from India have been published, this series attempts to define the clinical spectrum of patients from India. Methods: CT scan of the head and EEG were done ill all the 14 cases in addition to clinical history and detailed neurological examination. The diagnosis of GTS was made according to Tourette Syndrome Classification Study Criteria Group. Findings were statistically analyzed. Results: Ill all a total of 101 simple lllotor tics and 21 colllplex lllotor tics were observed ill 14 cases. Most common site of simple motor tics was the facio-nuchal region (170%) and type was eyebrow raising seen in 10 cases (71%). Commonest type of complex motor tics was nose blowing (35%). Vocal ties were found in all 14 cases, simple (156%), being more common than complex vocal tics i.e. coprolalia (42%). Throat clearing was found ill (50%) cases and was the most common type of simple vocal tic. Atcxiety was the commonest precipitating factor (70%), while family history was positive in 28%. OCD was seen in 64% and ADHD in 28%. All the 14 cases responded well to treatment. Conclusion: The clinical profile of patients ill our case series is similar to those reported from the western countries with the exception of coprolalia, and ADHD, the incidence of which is higher ill tiffs case series. 0860 Eye illOVelne[its in multiple system atrophy (MSA) T.J. Anderson1, N.P. Quillll2, L.M. Luxoll 3, A.M. Brollsteill4. 2Christchurch School of Medicine, University of Otago, Christchurch, New Zealand; SDepartment of Clinical Neurology, The National Hospitalfor Neurology and Neurosurgery, Queen Square, London, UI%" 3Department of Neuro-otology, The National Hospitalfor Neurology arid Neurosurgery, Queen Square, London, UK,"4Division of Neurosciences arid Mental Health, Medicine, Imperial College, London UK Background: Eye movement examination can be useful in the differ- ential diagnosis of parkinsonian syndromes. We determined to characterise the oculomotor features of multiple system atrophy (MSA). Method: We documented the clinical and laboratory oculomotor features in 30 patients with probable MSA (Qninn criteria) including four with subsequent post mortem confirmation. Results: Clinical examination revealed; excessive square wave jerks - 21/30 patients; mild vertical supranuclear gaze palsy - 8/30; Gaze evoked nystagmus - 12/30 patients, three of whom had no extraocular evidence of cerebellar dysfunction; positional downbeat llystagmus - 10/25; mild-moderate saccadic hypometria - 22/30; impaired smooth pursuit - 28/30; reduced VOR suppression - 16/24. Electro- oculography did not add significant new information. In four patients, subsequent post-lllortelll examination collfimled characteristic histo- pathological features of MSA. One of these had exhibited no clinical signs of cerebellar involvement (limb alld/or trullcal ataxia), whilst the other three had shown mild features. All had experienced some response to levodopa. None had gaze evoked nystagmus, but VOR supression was moderately impaired in the three patients in which this was examined. Down beat nysta~nus on positioning was present in three, including the one patient without clinical cerebellar involvement. Conclusion: Our findings suggest that the presence of impaired VOR suppression and downbeat nystagmus on positioning (Dix Hallpike maneuver) in particular are useful clues for the presence of MSA, even in the absence of (other) clinical cerebellar features. On the other hand, the presence of clinically slow saccades or, moderate or severe supralluclear gaze palsy, suggests a diagnosis other than MSA. 0861 Disorders of basal ganglia at the University Teaching Hospital Lusaka, Zambia Atadzhanov, M, Mwaba, P. University of Zambia, Lusaka, Zambia Objective: To determine tile prevalence and frequency of basal ganglia disorders ill adult black Zambian patients at the University Teaching Hospital (UTH) Lusaka, Zambia. Background: The Human Genome Project data showed that humans are identical across 99.9% of their genome. There is considerable evidence that despite being genetically identical, race/ethnicity appears to be all important factor ill the prevalence and clinical characteristics of lllally, if llot lllOSt, disorders. Data clearly shows that ethllicity has an essential role in the phenotypic expression of many basal ganglia disorders. Methods: Patients visiting the neuro-clinic and admitted to the University Teachillg Hospital with various neurological disorders ill the last 6 years (January 1999-Jalluary 2005) were analysed. We evaluated patients whose symptoms met the appropriate diagnostic criteria for basal ganglia disorders and experienced symptoms at least three days. Results: Records were available for 5783 patients and 202 (3.5?,';), with age range of 15-78, satisfied the criteria for basal ganglia disorders. The most frequent extrapyrmnidal syndromes (EPS) were: Parkinsoll's disease (31%), tremor (24%), chorea (20%), and dystonia' (16.5%). Myoclonus, tic, tardive dyskinesia, and other EPS (18.57,';) were rare in adult Zambian patients. In 25 patients (12.4%) EPS were manifesta- tions of HIV/AIDS. Conclusion: Basal ganglia diseases and disorders are not a frequent pathology ill Zambian adult patients. The results of our study suggests that ethnic differences have a profound effect on the clinical manifestations of EPS contrary to the general belief that environ- mental factors are the main factor. The presence of HIV/AIDS and its contribution to EPS disorders needs to be studied further. 0862 Panthothenate ldnase-associated neurodegeneration: a rare clinical phenotype Cebri~n E~; Rodriguez Alvarez JR~; Suarez Gil pa, Mourifio Sestelo MA~; Amigo MCa; Blanco-Arias P2. 1Neurology Dpt. Complejo Hospitalario de Pontevedra, Pontevedra, Spain; 2 ffundaciOn Pz~blicaGallega de Medicina GenOmica, Santiago de Compostela, Spain Introduction: Neurodegeneration with brain iron accumulation (Hallevorden-Spatz syndrome) includes a heterogeneous group of disorders differentiated by clinical, neuroimaging and molecular features. Recently it has been related with llmtatiolls ill gelle encoding pallthothellate killase 2 (PANK2). We report a case that shows atypical clinical manifestations

0859 Gilles de la tourtte's Syndrome- a case series from India

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Poster Abstracts Wednesday, November 9, 2005 $319

striatal haematoma. The patient became well after adjunction of haloperidol treatment.

The MRI showed the integrity of the corpus luysii ill both cases. Conclusion: Authors underline the rarity of hemiballismus due to all extraluysiall lesion and discuss its physiopathological, clinical, para- clinical and evolutionary aspects.

0859 Gilles De La Tourtte's Syndrome- A case series from India

Anand, KS 1, Garg, S 2, Gurtoo, A 3. 2Dr. Ram Manhoar Lohia Hospital, Delhi, India; 2Maulana Azad Medical College, Lok Nayak Hospital, Delhi, India; 3Lady Hardinge Medical College, Smt. Sucheta Kriplani Hospital, Delhi, India

Background: Gilles de la tourette"s Syndrome (GTS) is a neuro- psyclfiatric disorder characterized by motor and vocal tics associated with obsessive compulsive disorder (OCD) and attention deficit hyperactivity disorder (ADHD). Tiffs sylldrofme is often misdiagnosed as psychiatric disorder, therefore neuropsychiatric evaluation in patients suffering from it is very essential to make the correct diagnosis. Few reports, mostly single case reports of GTS from India have been published, this series attempts to define the clinical spectrum of patients from India. Methods: CT scan of the head and EEG were done ill all the 14 cases in addition to clinical history and detailed neurological examination. The diagnosis of GTS was made according to Tourette Syndrome Classification Study Criteria Group. Findings were statistically analyzed. Results: Ill all a total of 101 simple lllotor tics and 21 colllplex lllotor tics were observed ill 14 cases. Most common site of simple motor tics was the facio-nuchal region (170%) and type was eyebrow raising seen in 10 cases (71%). Commonest type of complex motor tics was nose blowing (35%). Vocal ties were found in all 14 cases, simple (156%), being more common than complex vocal tics i.e. coprolalia (42%). Throat clearing was found ill (50%) cases and was the most common type of simple vocal tic. Atcxiety was the commonest precipitating factor (70%), while family history was positive in 28%. OCD was seen in 64% and ADHD in 28%. All the 14 cases responded well to treatment. Conclusion: The clinical profile of patients ill our case series is similar to those reported from the western countries with the exception of coprolalia, and ADHD, the incidence of which is higher ill tiffs case series.

0860 Eye illOVelne[its in multiple system atrophy (MSA)

T.J. Anderson 1, N.P. Quillll 2, L.M. Luxoll 3, A.M. Brollsteill 4. 2Christchurch School of Medicine, University of Otago, Christchurch, New Zealand; SDepartment of Clinical Neurology, The National Hospital for Neurology and Neurosurgery, Queen Square, London, UI%" 3Department of Neuro-otology, The National Hospital for Neurology arid Neurosurgery, Queen Square, London, UK," 4Division of Neurosciences arid Mental Health, Medicine, Imperial College, London UK

Background: Eye movement examination can be useful in the differ- ential diagnosis of parkinsonian syndromes. We determined to characterise the oculomotor features of multiple system atrophy (MSA). Method: We documented the clinical and laboratory oculomotor features in 30 patients with probable MSA (Qninn criteria) including four with subsequent post mortem confirmation. Results: Clinical examination revealed; excessive square wave jerks - 21/30 patients; mild vertical supranuclear gaze palsy - 8/30; Gaze evoked nystagmus - 12/30 patients, three of whom had no extraocular evidence of cerebellar dysfunction; positional downbeat llystagmus - 10/25; mild-moderate saccadic hypometria - 22/30; impaired smooth

pursuit - 28/30; reduced VOR suppression - 16/24. Electro- oculography did not add significant new information. In four patients, subsequent post-lllortelll examination collfimled characteristic histo- pathological features of MSA. One of these had exhibited no clinical signs of cerebellar involvement (limb alld/or trullcal ataxia), whilst the other three had shown mild features. All had experienced some response to levodopa. None had gaze evoked nystagmus, but VOR supression was moderately impaired in the three patients in which this was examined. Down beat nysta~nus on positioning was present in three, including the one patient without clinical cerebellar involvement. Conclusion: Our findings suggest that the presence of impaired VOR suppression and downbeat nystagmus on positioning (Dix Hallpike maneuver) in particular are useful clues for the presence of MSA, even in the absence of (other) clinical cerebellar features. On the other hand, the presence of clinically slow saccades or, moderate or severe supralluclear gaze palsy, suggests a diagnosis other than MSA.

0861 Disorders of basal ganglia at the University Teaching Hospital Lusaka, Zambia

Atadzhanov, M, Mwaba, P. University of Zambia, Lusaka, Zambia

Objective: To determine tile prevalence and frequency of basal ganglia disorders ill adult black Zambian patients at the University Teaching Hospital (UTH) Lusaka, Zambia. Background: The Human Genome Project data showed that humans are identical across 99.9% of their genome. There is considerable evidence that despite being genetically identical, race/ethnicity appears to be all important factor ill the prevalence and clinical characteristics of lllally, if llot lllOSt, disorders. Data clearly shows that ethllicity has an essential role in the phenotypic expression of many basal ganglia disorders. Methods: Patients visiting the neuro-clinic and admitted to the University Teachillg Hospital with various neurological disorders ill the last 6 years (January 1999-Jalluary 2005) were analysed. We evaluated patients whose symptoms met the appropriate diagnostic criteria for basal ganglia disorders and experienced symptoms at least three days. Results: Records were available for 5783 patients and 202 (3.5?,';), with age range of 15-78, satisfied the criteria for basal ganglia disorders. The most frequent extrapyrmnidal syndromes (EPS) were: Parkinsoll's disease (31%), tremor (24%), chorea (20%), and dystonia' (16.5%). Myoclonus, tic, tardive dyskinesia, and other EPS (18.57,';) were rare in adult Zambian patients. In 25 patients (12.4%) EPS were manifesta- tions of HIV/AIDS. Conclusion: Basal ganglia diseases and disorders are not a frequent pathology ill Zambian adult patients. The results of our study suggests that ethnic differences have a profound effect on the clinical manifestations of EPS contrary to the general belief that environ- mental factors are the main factor. The presence of HIV/AIDS and its contribution to EPS disorders needs to be studied further.

0862 Panthothenate ldnase-associated neurodegeneration: a rare clinical phenotype

Cebri~n E~; Rodriguez Alvarez JR~; Suarez Gil pa, Mourifio Sestelo MA~; Amigo MCa; Blanco-Arias P2. 1Neurology Dpt. Complejo Hospitalario de Pontevedra, Pontevedra, Spain; 2 ffundaciOn Pz~blica Gallega de Medicina GenOmica, Santiago de Compostela, Spain

Introduction: Neurodegeneration with brain iron accumulation (Hallevorden-Spatz syndrome) includes a heterogeneous group of disorders differentiated by clinical, neuroimaging and molecular features. Recently it has been related with llmtatiolls ill gelle encoding pallthothellate killase 2 (PANK2).

We report a case that shows atypical clinical manifestations