ATYPICAL MANIFESTATIONS OF SUBACUTE SCLEROSING PANENCEPHALITIS: CASE SERIES

Subacute Sclerosing Panencephalitis (SSPE) is a rare, progressive degenerative disease of the brain caused by reactivation of aberrant measles virus. Classical symptoms of SSPE are described as behavioral changes, declining scholastic performance, myoclonic jerks etc. However, at times it presents with varied atypical manifestations, which makes diagnosis dif?cult. Hereby we report two patients with unusual manifestations of SSPE. In ?rst case, 14-year-old boy presented with recurrent falls of 9 months duration. Initially, it was 1-2 times per week, progressing over 9 months to frequency of 40-50 falls per day. In second case, 21-year-old gentleman presented with behavioral changes and memory disturbance of 1-year duration. However, his family members noticed worsening of symptoms 1 month before presentation as he started developing involuntary jerky movements involving the right upper and lower limb. In both of them electroencephalogram (EEG) was showing long interval periodic discharges and cerebrospinal ?uid was showing elevated titers of anti-measles antibody overall suggesting SSPE. Hence it needs much attention for atypical presentations and also considering SSPE in differential diagnosis of unsolved atypical neurological presentations.

Callosotomía posterior: un nuevo abordaje en Cirugía de Epilepsia / Posterior callosotomy: a new approach in Epilepsy Surgery

Objetivo: describir la nueva técnica de callosotomía posterior utilizada en cirugía de epilepsia, junto a sus indicaciones, ventajas comparativas y sus resultados. Métodos: se muestran en secuencias de video dos casos de callosotomía posterior, en pacientes con epilepsia refractaria severa, con crisis tipo "drop attacks" (pérdida súbita del tono postural), presentando traumatismos a repetición, con potencial riesgo de vida. Ambos pacientes, una mujer de 35 años y un varón de 24 años de edad, presentaban una malformación congénita del desarrollo cortical denominada doble corteza. Un caso fue operado en posición semisentada, con abordaje parafalcino derecho, el segundo en decúbito ventral, con abordaje parafalcino izquierdo. Se realizó una incisión vertical parasagital de 7cm, a 2 cm de la línea media, con límite inferior en el inion y superior en la unión de la sutura sagital y lambdoidea. Se realizó una craneotomía de 5 x 5cm, exponiendo el seno longitudinal superior. La anatomía venosa de la región permite sin inconvenientes el desplazamiento lateral occipito-parietal. Se realizó una callosotomía que incluyó esplenio y cuerpo llegando anteriormente al sector que une ambas áreas motoras suplementarias. Resultados: no se presentaron complicaciones postoperatorias. El síndrome de desconexión apareció en un caso, desapareciendo en un periodo de 2 semanas. Se obtuvo una reducción del 90% de crisis, mejorando la calidad de vida y otorgando un mayor grado de independencia a ambos pacientes. Conclusión: la callosotomía posterior es un procedimiento seguro, poco invasivo y altamente efectivo contra las crisis de drop attacks en epilepsias refractarias

Objective: to describe the new technique of posterior callosotomy applied to epilepsy surgery, including its indication, comparative advantages and results. Methods: two surgical cases are presented on video sequences, including patients with refractory epilepsy, with "drop attack" type seizures (loss of postural tone), presenting with recurring trauma and a potential life risk. Both patients, a 35 years-old woman and a 24 years-old man suffered from a congenital malformation of cortical development known as double cortex. One of the cases was operated in a semi-sitting position, trough a right parafalcine approach, and the other one in a prone position trough a left parafalcine approach. A 7cm parasagittal vertical incision, 2cm lateral to midline was performed, considering its upper limit at the union of the lambdoid and parasagittal suture, and its lower limit at the inion. A craniotomy of 4 by 4cm was performed, exposing the superior sagittal sinus. The venous regional anatomy allows an easy parieto-occipital lateral retraction. The callosotomy included the splenium and the body reaching anteriorly up to the region where we find both supplementary motor areas. Results: no postoperative complications were found. One case presented with a disconnection syndrome, resolving in 2 weeks. A 90% reduction of the seizure frequency was achieved, improving the quality of life and the degree of Independence of the patients. Conclusions: posterior callosotomy is a safe, less invasive and highly effective procedure against drop attack type seizures in refractory Epilepsy

Intracerebral Mass Lesions Presenting "Drop Attacks"

"Drop attack" is defined as sudden falling spell occurring without warning or postictal symptoms with immediate righting, and without loss of consciousness. Numerous mechanism have been proposed to explain the drop attacks. But there were no scientifically established answer for the extraordinary rapid evolution of the drop attacks. The authors report three cases of intracerebral mass lesions presenting with drop attacks. Among them, one was racemose brain abscess on the juxtaventricle of right parieto-occipital area in 3-year old girl, the another one was germinoma at genu portion of corpus callosum and pineal gland respectively in 20-year old male, and the remained one was neurocysticercosis around frontal horn of lateral ventricle in 60-year old male. The brain abscess and neurocysticercosis were almost totally removed, and conventional radio-therapy after stereotatic biopsy was carried out to germinoma of corpus callosum and pineal region. Drop attacks symptoms were abolished immediately after mass removal in brain abscess and neurocysticercosis, and gradually subsided in the germinoma case.

Therapeutic effects observation of endovascular stenting on the patients with drop attacks caused by carotid artery or vertebra-basilar artery stenosis(report of 2 cases) / 临床神经病学杂志

Objective To observe the therapeutic effects of endovascular stenting on the patients with drop attacks caused by carotid artery or vertebra-basilar artery stenosis.Methods Two drop attacks patients,who were diagnosed with symptomatic carotid artery or vertebra-basilar artery stenosis by DSA,were performed endovascular stenting.Case one had four serious artery stenoses at basilar artery,left vertebra artery and subclavian artery.Case two had double internal carotid artery.Six stents were placed,and filter protection devices were used in case two.After operating,the flowing up was performed for nine months or two years.Results The clinical symptoms of two patients were disappeared after operation.Case one performed flowing up for 9 months after operation,but he died by infarction of brain stem for his irregular taking medicine for 3 months since 6 months after operation.During flowing up for 2 years,case two was no relapse and no restenosis by DSA peoformed at 1 year after operating.Conclusions Endovascular stenting is a safe and efficient method for treating the patients with drop attacks caused by carotid artery or vertebra-basilar artery stenosis.