S336 19th World Congress of Neurology, Poster Abstracts / Journal of the Neurological Sciences 285 S1 (2009) S155–S339

defecation, excessive sweating and heat intolerance. Two days

prior to presentation, she suddenly stopped walking. Examination

revealed a young woman with fever of 38.5, bilatral proptosis and

pitting pedal edema. She had goitre with warm sweaty palms and

fine tremors. Power was grade 1 in all the limbs. The deep tendon

reflexes were exaggerated with sustained ankle clonus. She had

bilateral proptosis, lid lag and lateral rectus palsy. The pulse rate was

144beats/min. The blood pressure was 170/50. Potassium level was

2mmols/L. An assesment of thyrotoxic periodic paralysis was made.

She improved remarkably after rehydration. Power increased to

grade 4 with mainly proximal myopathy. While being investigated,

she went into premature labour and developed thyroid storm. She

died in labour ward.

Conclusion: Thyrotoxic hypokalemic periodic paralysis is unusual

in the African woman. The diagnosis and management of such a

patient present great challenges in a resource poor setting.

PO33-FR-07

Centropontine myelinolysis and hypokalemia

I. Ben Hamouda1, M.N. Tougourti2, D. Mohsen2, M. Hamza2.1Neurology, Charles Nicolle Hospital, Tunis, Tunisia; 2Medicine, Razi

Hospital-La Manouba, Manouba, Tunisia

Purpose: Centropontine myelinolysis is a rare but severe anatomo-

clinical entity. Fast normalisation of severe hyponatremia has long

been considered as the main cause of this injury. Hypokalemia has

rarely been implicated in the pathogenesis of this disease. We report

a centropontine myelinolysis case with chronic and permanent

hypokalemia.

Observation: A 42 years old woman is hospitalized for numbness

and weakness in the lower limbs. Medical history: mild hypothy-

roidism and Sjogren’s syndrome, diagnosed a few years ago, initially

presented as a tubular nephritis with permanent hypokaliemia.

Renal function and natremia were normal. On admission, she

was alert and had tetrapyramidal syndrome. Cranial nerves were

preserved. Biological investigation showed a severe hypokalemia

(2.6mmol/l). Cerebral MRI disclosed a central pontine hypersignal

T2 suggestive of myelinolysis. Angio MRI excluded active vasculitis.

Discussion: Central pontine myelinolysis, once a merely anatomic

diagnosis, is currently easier to diagnose. In our opinion, fast

normalisation of hyponatremia should no longer be considered as

its main cause, as many other conditions have been implicated in its

aetiology, such as alcoholism, anorexia nervosa, chronic renal and

liver diseases. Chronic hypokalemia is presumably the cause of pon-

tine injury in our patient. Six other similar cases have been reported

in the literature. In some of them, hpokalemia was of acute onset

and transitory. In one observation, hypokalemia was secondary to a

tubular nephritis in the setting of Sjogren’s syndrome, like our case.

The mechanism of the pontine injury is unknown. Some hypotheses

are proposed referring to cellular injuries described in some

pathological conditions and presumably caused by hypokalemia.

Conclusion: The increasingly available neuroimaging techniques

enable an easier diagnosis of centropontine myelinolysis, which

clinical presentation, treatment and prevention are linked to its

different etiologies.

PO33-FR-08

Hyponatraemia – role of high environmental temperature

and salt intake

P. Sanchetee. Sanchetee Neurology Research Institute, Jodhpur, India

Purpose: Hyponatraemia, defined as serum sodium concentration

less than 135 mEq/L, is the commonest electrolyte abnormality

seen in neurocritical care. Risk factors include extremes of age,

postoperative, malignancy, pulmonary disease, pharmacological

agents, and patients with brain injury or infection. Aim of this

presentation is to document hyponatraemia in the setting of

restriction in salt intake, use of diuretics and routine use of

hypotonic fluids in hot climate.

Methods: Observational study over a period of 15 months of

23 patients presented with severe hyponatraemia and with

encephalopathy complicating acute neurological presentation.

Results: There were 14 male and 9 female patients in age range

of 33 years to 71 years. Underlying disorders were stroke (6),

CNS infections (4), status epilepticus (3), head injury (3), primary

hyponatraemia and miscellaneous (7). Associated factors were

restriction of salt intake (16) and use of diuretics (9), use of

hypotonic intravenous fluid replacement (11), high environmental

temperature (15) and no cause evident in 3 patients. Clinical

presentation was nonspecific and included altered sensorium, ab-

normal behavior, seizures, fever, and bradycardia. While 7 patients

died and 5 were severe disabled, 10 patients made good clinical

recovery. Prognostic factors were underlying disorder, coma at

onset, advancing age, and duration and severity of hyponatraemia.

Conclusions: Hyponatraemia is a common following acute

neurological insult. There is need to re-evaluate the practice of

salt restriction and use of diuretics in patients with hypertension

in regions with high ambient temperature. Awareness and early

treatment can decrease risk of death and length of hospital stay.

PO33-FR-09

Case series of hyperglycemia presenting as fit or funny

movements

P. Alagia Nambi1, V. Karthikeyan2, M. Dhiviya3,

P. Namasivayam Balamurugan3, R. Thamilselvi4. 1Medicine,

Sri Gokulam Hospital, Salem, India; 2Nephrology, Sri Gokulam

Hospital, Salem, India; 3Neurology, Sri Gokulam Hospital, Salem,

India; 4Sri Gokulam Hospital, Salem, India

Nonketotic Hyperosmolar Hyperglycemia (NKHH) is characterized

by extreme hyperglycemia, increased osmolarity and severe

dehydration without significant Ketosis or acidosis. We report a

case series of NKHH presenting as either recurrent focal seizures

or abnormal movements.

A total of 7 patients were documented by video recording over 3

yrs. All presented as focal seizures or abnormal movements and

all were fully conscious either during (n =4) or in between attacks

(n = 3). Table 1 shows their biochemical profile compared with

typical DKA and NKHH. In all CT brain were normal and their EEG

showed either focal or generalized slowing or sharp waves.

Our patients were between classical DKA and NKHH in terms of

their blood biochemistry but clinically they had the following

typical prototype: Highly recurrent seizures or episodes of

movements 20–50/day. Normal Neuro Imaging and all were

symptom free with fluid and insulin therapy. Early recognition

of neurological manifestation of non ketotic hyperglycemia can

prevent the mortality associated with these syndromes.

PO33-FR-10

Impaired mitochondrial energy metabolism and neuronal

apoptotic cell death after chronic aluminum exposure in rat

brain

P.O.O. Khanna, B.I.M. Nehru. Dept of Biophysics, Panjab University,

Chandigarh, India

The present study elucidates a possible mechanism by which

chronic aluminum exposure (100mg/kg b.wt. p.o. for 8 weeks

daily) causes neuronal degeneration. Mitochondria, the primary

site of cellular energy generation and oxygen consumption might

represent a likely target for aluminum toxicity. Therefore, the

objective of the current study was to investigate the effect of chronic

aluminum exposure on mitochondrial energy metabolism, oxidative

stress generation and its implication in the induction of neuronal

apoptosis in rat model. Mitochondrial fractions were isolated from

cerebral cortex, mid brain and cerebellar region of rat brain and

the complexes were estimated biochemicaly in the same fractions.

Our results indicated significant decrease in the activity of complex

I, II and IV in all the three regions, though the changes were more

19th World Congress of Neurology, Poster Abstracts / Journal of the Neurological Sciences 285 S1 (2009) S155–S339 S337

pronounced in mid brain region. The MTT assay which indirectly

shows the activity of complex III did not register any significant

change except for mid brain. Mitochondria generate ROS that are

thought to augment intracellular oxidative stress. The alterations in

the mitochondrial electron transfer enzyme activities in turn might

have caused an increase in malondialdehyde which is observed

in all the three regions. Further the decreased levels of GSH and

also decreased MnSOD activity in the mitochondrial fraction of rat

brain enhanced oxidative stress. Thus, chronic aluminum exposure

increases the oxidative stress both by mitochondrial damage as

well as due to the failure in the ROS removal system which in

turn causes oligonucleosomal DNA fragmentation and formation

of comet, a hallmark of apoptosis. The present study provides an

evidence of impaired mitochondrial bioenergetics and apoptotic

neuronal degeneration after chronic exposure of aluminum.

PO33-FR-11

Protective role of lithium during aluminium-induced

alterations in rat brain

P. Bhalla, M.L. Garg, D.K. Dhawan. Department of Biophysics, Panjab

University, Chandigarh, India

The present study was conducted to assess the role of lithium in

aluminium toxic conditions. To carry out the various investigations,

Al was administered at a dose of 100mg/kg b.wt./day whereas

lithium was supplemented in diet (1.1 g/kg diet, daily) for the

period of two months. Ca2+ ATPase activity was observed to be

decreased in Al treated animals. Whereas, a significant increase in

Ca2+ influx and in the levels of cAMP were observed following Al

treatment which were decreased with lithium co-administration.

Further, a significant increase in the levels of phospholipase C

(PLCyI) was observed following Al treatment which was normalized

following lithium supplementation. The nitric oxide synthase

enzyme activity and the levels of L-citrulline were also found to

be significantly increased in cerebrum and cerebellum after Al

treatment, which were significantly decreased following lithium

supplementation. The DNA damage caused in the cell as a result

of Al treatment was examined by single cell gel electrophoresis

as well as DNA fragmentation studies and a significant increase

in the DNA damage was observed which was found to be

significantly improved upon lithium supplementation. Alterations

in the neuronal histoarchitecture and ultra structure were also

observed following Al treatment. Lithium supplementation greatly

restored normalcy in the cerebrum and cerebellum layers with

no loss of cerebral or purkinje cell layer as evident by light

microscopy. Further, lithium supplementation to Al treated rats

resulted in appreciably thwarting the ultrastructural changes with

regard to integrity of the cells as a whole as well as the cell

organelles as observed by transmission electron microscopy. The

study demonstrates that lithium, has the potential in containing

or reversing the Al-induced functional and structural changes as

evidenced by oxidative stress, DNA damage and altered calcium

homeostasis as well as disordered signal cascade.

PO33-FR-12

Sialidosis and malignancy: the possible relationship between

the abnormal metabolism of sialitic acid and malignancy from

a family of genetically defined sialidosis

T. Kobayashi1, Y. Yagi1, A. Machida1, S. Touru1, T. Uchihara2.1Neurology, Nakano General Hospital, Tokyo, Japan; 2Neurology, Tokyo

Metropolitan Institute for Neuroscience, Tokyo, Japan

Purpose: To investigate the possible relationship between the

abnormal metabolism of sialitic acid and malignancy from a family

of genetically defined sialidosis.

Methods: Clinical and pathological studies of three siblings of

the family of sialidosis type I carrying V217M/G243R mutations

in sialidase gene.

Results: All three siblings (elder and younger sisters, and brother)

were biochemically identified marked sialidase deficiency. The

genetical analysis was performed from cultured fibroblasts of

the elder sister (Naganawa et al., 2000). The clinical onsets of

their sialidosis were ataxic gaits or incidentally identified macular

cherry-red spot around age 15. Their clinical symptoms had been

gradually worse with painful convulsive attacks, action myoclonus

and impairment of walking, but their intelligence were normal.

The elder sister suffered from colon cancer at age 45. And the

younger sister suffered from ovarian tumor at age 37. The brother

suffered from malignant lymphoma of diffuse B cell type at age 30,

which was refractory to multiple courses of standard (CHOP)

and intensified (ESHAP) chemotherapy and local irradiation for

palliation.

Discussions and Conclusions: Association of malignancy (malig-

nant lymphoma, colon cancer, and ovarian tumor) has not been

described in the literature and could be by chance. However, a

series of evidence suggests a possible link between sialidase/sialic

acid and malignancy (Miyagi et al., 2004). It is interesting that

less expression of sialidase in some cell lines of colon carcinoma

is related to their highly metastatic nature relative to cell lines

expressing more abundant sialidase (Sawada et al, 2002). It is also

reported that the presence of sialitic acid in the surface of diffuse

large B cell lymphoma is associated with shorter survival (Suzuki

et al, 2003). So this family provides a quite valuable information of

investigating between malignancy and sialidase deficiency/sialitic

acid metabolism.

PO33-FR-13

Inhibitory effect of tyrphostin AG126 on brain synaptosomal

dysfunction induced by cholesterol oxidation products

D.E. Kim1, C.K. Ha2, S.H. Park3, J.K. Roh4. 1Neurology, Seoul Veterans

Hospital, Seoul, Republic of Korea; 2Neurology, Inha University

Hospital, Incheon, Republic of Korea; 3Neurology, Seoul National

University Bundang Hospital, Bundang, Republic of Korea; 4Neurology,

Seoul National University Hospital, Seoul, Republic of Korea

Background: Formation of cholesterol oxidation products is a

suggested mechanism of neurodegenerative disorders. Neuronal

cell death is mediated by an increased release of excitotoxic gluta-

mate from the presynaptic nerve endings. Tyrosine-specific protein

kinases modulate neurotransmitter release at the nerve terminals.

Tyrphostin AG126 has anti-inflammatory and cytoprotective effects.

However, it remains uncertain whether tyrphostin AG126 has an

effect preventing alteration of nerve terminal function induced by

the cholesterol oxidation products.

Methods: The present study was performed to assess the effect

of cholesterol oxidation products against nerve terminal function

using synaptosomes isolated from rat cerebrum. We determined

the preventive effect of tyrphostin AG126 against oxysteroltoxicity

by measuring the effects on the glutamate release, depolarization

of membrane potential, changes in Ca2+ levels, and Na+/K+-ATPase

activity.

Results: Synaptosomes treated with 7-ketocholesterol or 25-

hydroxycholesterol exhibited a sustained release of glutamate,

depolarization of membrane potential, early rapid increase in

cellular Ca2+ levels and decrease in Na+/K+-ATPase activity. Those

responses were concentration-dependent. Treatment of tyrphostin

AG126 interfered with alteration of synaptosomal functions and

decrease in Na+/K+-ATPase activity induced by 7-ketocholesterol or

25-hydroxycholesterol.

Conclusions: The results show that 7-ketocholesterol and 25-

hydroxycholesterol seem to cause the release of glutamate by

inducing depolarization of membrane potential and early rapid

increase in cellular Ca2+ levels and by inactivating Na+/K+-ATPase

in the cerebral synaptosomes. Treatment of tyrphostin AG126 may

prevent the oxysterol-induced nerve terminal dysfunction.