[Symptomatic trigeminal autonomic cephalalgia without headache]. / Szimptómás trigemino-autonóm tünetcsoport fejfájás nélkül.

We report the case of a 60-year-old man who exhibited trigeminal autonomic symptoms on his right side (numbness of the face, reddening of the eye, nasal congestion) occurring several times a day, for a maximum of 60 se-conds, without any pain. The complaints were similar to trigeminal autonomic cephalalgia, just without any headache. Our 60-year-old male patient underwent a craniocervical MRI as part of his neurological workup, which revealed lesions indicative of demyelination. Further testing was guided (ophthalmological examination, VEP, CSF test) by the presumptive diagnosis of multiple sclerosis. It is likely that in his case the cause of these trigeminal and autonomic paroxysms is MS. Here we present an overview of the few cases we found in the literature, although we did not find any similar case reports. Perhaps the most interesting among these is one in which the author describes a family: a 54-year-old female exhibiting the autonomic characteristics of an episodic cluster headache, only without actual headache, her son, who had typical episodic cluster headaches with autonomic symptoms, and the woman's father, whose short-term periorbital headaches were present without autonomic symptoms. We had not previously encountered a case of trigeminal autonomic cephalalgia without headache in our practice, nor have we had an MS patient exhibiting similar neurologic symptoms. The significance of our case lies in its uniqueness.

[TRANSIENT PSEUDOBULBAR SYNDROME IN UNILATERAL FRONTAL OPERCULAR INFARCTS]. / ÁTMENETI PSEUDOBULBARIS TÜNETCSOPORT EGYOLDALI FRONTÁLIS OPERCULARIS INFARKTUSOKNÁL.

The classic anterior (frontal) opercular syndrome (Foix-Chavany-Marie sy.) is a cortical pseudobulbar palsy mainly due to bilateral lesions of anterior brain operculum. In 2000 the authors had a 70-year old female patient with acute onset of swallowing and speaking difficulty. Neurological examination established a left facial central palsy, the palsy of the tongue and the soft palate, dysarthry, difficulty in chewing with left side hemiparesis. The CT scan showed a right side (one-sided) frontal opercular ischemic lesion. This event switched their attention especially to this group of cases and subsequently the authors collected 12 patients with these symptoms. Authors discuss the patomechanism of transient pseudobulbar palsy that occurs due to unilateral opercular lesion that the diaschisis effect might explain.

[Big blind spot syndrome (papillophlebitis) with unusual visual field defect]. / Nagy vakfolt szindróma (papillophlebitis) szokatlan látótérkieséssel.

We present three cases, where young patients had unilateral disc edema with normal optic nerve function. We diagnosed their disease as big blind spot syndrome (BBSS). What is remarkable, however, is that in two of the three cases the extent of the visual field defect considerably exceeded the one regularly emerging in BBSS, which caused us some difficulty in differential diagnosis. The characteristics and the differential diagnostic questions of the big blind spot syndrome are summarised and we would like to draw attention to this unusual, but probably not very rare, form of it.

[Pseudo abducens palsy]. / Pseudoabducens paresis.

In this study, we present two cases of different eye movement disorders with variable case histories but with the same end stage; abduction paresis of one of the eyes, which ceased when the other eye was covered. Our differential diagnosis is that either the ocular form of myasthenia gravis, convergence spasm or ocular myotonia could explain the symptoms. However, we hypothesize that the clinical picture corresponds to pseudo abducens palsy or focal dystonia of the extraocular muscle, which in turn could be the result of impaired inhibition of the tonic resting activity of the antagonistic medial rectus muscle. We offer an explanation for the patomechanism of pseudoabducens palsy and the variants of internuclear ophthalmoplegia.

[Posterior cortical atrophy (Benson-syndrome)]. / Hátsó corticalis atrophia (Benson-szindóma).

We present the characteristics of posterior cortical atrophy--a very rare cortical dementia--in a 69 year old woman's case. Our patient's symptoms began with a visual problem which was initially explained by ophthalmological disorder. After neurological exam visual agnosia was diagnosed apart from other cognitive disorder (alexia without agraphia, acalculia, prosopagnosia, constructional disorder, clock-time recognition disorder, dressing apraxia, visuospatial disorientation). The brain MRI showed bilateral asymmetric parieto-occipital atrophy which is characteristic of posterior cortical atrophy.

[Posterior ischaemic optic neuropathy]. / Hatsó ischaemiás opticusneuropathia.

Here one case report of the posterior ischaemic optic neuropathy, a rare and underdiagnosed form of the non arteritic ischaemic optic neuropathy is presented, to underline the value of the MRI in the diagnosis. The ischaemic optic neuropathy is the infarction of the optic nerve. Depending on the affected segment of optic nerve (optic nerve head or retrobulbar segment) two subclasses exist: the anterior (AION) and the posterior (PION) ischaemic optic neuropathy. Ischaemic optic neuropathy characterized by sudden, painless, mononuclear loss of vision, and/or visual field defect, that is accompanied by a diagnostic picture of the optic disc fundus only in the case of the AION. The diagnosis of the PION is based on a diagnosis of exclusion described by Hayreh in 1981. The macular and retinal lesions, the etiological role of toxic agent, the compression and the inflammation of the optic nerve all have to be excluded. The differential diagnosis between the PION and the retrobulbar neuritis is more difficult. Nowadays, in addition to the case history and the clinical data the diagnosis of the PION could be confirmed with help of VEP (visual evoked potential) and MRI. In the case of an old woman who had a sudden, painless visual loss of left eye we confirmed the diagnosis of PION with MRI which was presumed after had excluding other etiological factors.

[Uncommon manifestation of central nervous system sarcoidosis]. / A sarcoidosis ritka idegrendszeri manifesztációi.

Two cases of uncommon manifestation of central nervous system sarcoidosis are reported. A 42 year-old man had a spinal cord sarcoidosis. MRI of the spinal cord showed myelopathy in the cervico-thoracic region, and the T2-weighted image showed increasing signal intensity. Neurological symptoms did not correlate with radiological abnormalities. Neurological manifestation was paucisymptomatic. Half a year later steroid and azathioprine therapy led to almost complete radiological and clinical regression. In the second case we present a 49 year-old woman who had left side internuclear ophthalmoplegia and the brainstem lesion. The patient was proven to have sarcoidosis. In this case no abnormalities were found in brain MRI. Neurological symptoms could not be detected by MRI, probably caused by brainstem parenchymal lesions consisting of microgranulomatosis that is sarcoid "brainstem encephalitis". Neurological symptoms improved after steroid treatment in this case too. In both of the cases pulmonary lymphadenopathy helped to diagnose sarcoidosis. In our cases there were interesting correlations between neurological symptoms and MRI abnormalities. At the spinal cord sarcoidosis the radiological abnormalities were more striking than the clinical manifestation. In the other case we found distinct brainstem symptoms but could not detect MRI abnormalities.