Clinical Neuroscience

[In memoriam professor Klára Majerszky]

CSIBA László, VÁRADY Géza

JULY 30, 2008

Clinical Neuroscience - 2008;61(07-08)

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[IDIOPATHIC TOLOSA-HUNT SYNDROME: FOUR ADDITIONAL CASES]

KIRBAS D, TOPCULAR B, OZCAN ME, KARAGOZ Sakalli N, GUL G, KALYONCU Aslan I

[Idiopathic Tolosa-Hunt syndrome (ITHS) is a very rare cause of painful ophthalmoplegia characterized by unilateral orbital pain, ipsilateral oculomotor paralysis and prompt response to steroids. In this paper we report 4 additional cases of ITHS. This rare cause of painful ophthalmoplegia effects the cranial nerves due to a granulomatous lesion of unknown etiology in the cavernous sinus or superior orbital fissure. The International Headache Society redefined the diagnostic criteria for ITHS but it is still mostly a diagnosis of exclusion. Careful evaluation and follow-up is essential for diagnosis. Optimal therapy duration and dosage and prophylactic treatment in recurrent cases needs further research.]

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[BRAIN LATERALIZATION AND SEIZURE SEMIOLOGY: ICTAL CLINICAL LATERALIZING SIGNS]

HORVÁTH A. Réka, KALMÁR Zsuzsanna, FEHÉR Nóra, FOGARASI András, GYIMESI Csilla, JANSZKY József

[Clinical lateralizing signs are the phenomena which can unequivocally refer to the hemispheric onset of epileptic seizures. They can improve the localization of epileptogenic zone during presurgical evaluation, moreover, their presence can predict a success of surgical treatment. Primary sensory phenomena such as visual aura in one half of the field of vision or unilateral ictal somatosensory sensation always appear on the contralateral to the focus. Periictal unilateral headache, although it is an infrequent symptom, is usually an ipsilateral sign. Primary motor phenomena like epileptic clonic, tonic movements, the version of head ubiquitously appear contralateral to the epileptogenic zone. Very useful lateralization sign is the ictal hand-dystonia which lateralizes to the contralateral hemisphere in nearly 100%. The last clonus of the secondarily generalized tonic-clonic seizure lateralizes to the ipsilateral hemisphere in 85%. The fast component of ictal nystagmus appears in nearly 100% on the contralateral side of the epileptic focus. Vegetative symptoms during seizures arising from temporal lobe such as spitting, nausea, vomiting, urinary urge are typical for seizures originating from non-dominant (right) hemisphere. Ictal pallor and cold shivers are dominant hemispheric lateralization signs. Postictal unilateral nose wiping refers to the ipsilateral hemispheric focus compared to the wiping hand. Ictal or postictal aphasia refers to seizure arising from dominant hemisphere. Intelligable speech during complex partial seizures appears in non-dominant seizures. Automatism with preserved consciousness refers to the seizures of non-dominant temporal lobe.]

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[SUCCESSFULL SURGICAL REMOVAL OF A MESENCEPHALIC CAVERNOUS ANGIOMA, WHICH WAS RESPONSIBLE FOR PROGRESSIVE NEUROLOGICAL DEFICITS]

ZSOLDOS Tamás, MOLNÁR Anna, JÁNOSSY Ágota, KUNCZ Ádám, NAGY Ernő, DEÁK Gábor, BARZÓ Pál

[Cavernous angiomas comprise 5-10% of all vascular malformations in the central nervous system, occuring most frequently in the supratentorial region, and 20% of them in the brain stem. According to literature, brain stem cavernous angiomas occur most frequently in the pons (60%), and equally in the mesencephalon (20%) and in medulla oblongata. In clinical evaluation the authors describe the successful removal of a mesencephalic cavernous angioma causing progressive neurological deficits and symptoms. The authors present a case of a 51 year old female, who had developed 1 year prior to her admittance: fatigue, weakness in the right upper limb and fingers, right lower limb ataxia. One month later, her lower right limb developed sensory deficits. The first neurological exploration indicated dysarthria, moderate facial and right hemiparesis, hemihypaesthesia and ataxia. CT and MR imaging indicated multilobulated cavernomas in the mesencephalon. After conservative treatment the patient became almost symptom free, and thus neurosurgical treatment was not discussed. Later on her symptoms fluctuated, but after 6 month she suddenly developed progressive right hemiparesis, right facial weakness, serious dysphasia, and emotional incontinence combined with continuous spastic sobbings. The controll MRI showed enlargement of the cavernomas and new extravasation. Surgery was indicated for removing the cavernomas. The left infratentorial, supracerebellar approach revealed a blood engorged cavernoma in the center of the mesencephalon, almost dividing it. The cavernomas and accompanying haematoma was exstirpated. The patient's neurological symptoms rapidly improved after surgery, her dysphasia as well as motor weakness have disappeared. Six days after surgery, we discharged a neurologically symptomless and self-supporting patient. The literature and the presented case indicates that the correct timing and proper surgery allows brain stem cavernomas to be safely removed, or significantly bated, which results in the massive regression of neurological symptoms.]

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