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A previously healthy 30-year-old woman who had cognitive impairment since childhood suddenly developed progressive spastic paraparesis. Visual impairment and characteristic retinal macular spots supported the diagnosis of Kjellin syndrome. This disease, probably transmitted by autosomal recessive inheritance, is seldom observed in clinical practice. We describe the characteristics of Kjellin syndrome and the differential diagnosis, including other macular changes associated with spastic paraparesis.

Ictal SPECT is a valuable method for the presurgical exploration of partial epilepsy. (99m)Tc Ethyl Cysteinate Dimer stable during 6h has contributed to develop ictal studies to evaluate the location of partial seizure. The aim of this study was to investigate some factors playing a possible role on the regional increase of cerebral blood flow (rCBF) Twenty-three patients with refractory partial epilepsy (temporal epilepsy n=16, extratemporal epilepsy n=7) were included. All the patients underwent a presurgical evaluation (phase I) during a week with detailed clinical history, cranial magnetic resonance imaging, monitoring EEG and video. Ictal and interictal SPECT were performed using a fast rotating brain dedicated camera (TOMOMATIC 564) in a quiet and normally illuminated room with controlled EEG (interictal) or video EEG (ictal); Scanning was started one hour after injection of ECD Tc administered in IV a few seconds after the electrical onset seizure. Slices parallel to the long axis of the temporal lobe were reconstructed. SPECT images were evaluated after normalisation. This study shows that

The authors report the case of a 37 years old woman with no previous medical history, admitted to hospital for investigation of unexplained syncope, sometimes associated with generalised fits. After standard non-invasive cardiovascular investigations, no diagnosis could be made. The tilt test induced a minor syncopal episode without reproducing the clinical symptoms. In view of the discordance between the induced and spontaneous symptoms, a neurological opinion was requested. During the recording of an electroencephalogram, syncopal atrioventricular block was observed, preceded by auditory hallucinations, reproducing exactly the clinical symptoms. Analysis of the sequence of events showed the conduction defect to arise after the onset of the epileptic fit, indicating a diagnosis of syncopal complete atrioventricular block complicating cryptogenic temporal epilepsy, and requiring specific treatment. This case illustrates the importance of close collaboration between cardiologists and neurologists in the management of cases of unexplained syncope.

We report a case of rubral infarct involving adjacent ipsilateral thalamic structures in a 54-year-old man with hypertension and coronary heart disease. Clinical picture at onset was suggestive of Weber's syndrome leading to a Claude's syndrome with vertical gaze palsy and sleep disturbances. Single photon emission computed tomography using Hm-PaO-Tc revealed a crossed cerebellar diaschisis. This is, to our knowledge, the first case of such crossed cerebellar diaschisis in a patient with rubral infarction.

We report a case of adrenomyeloneuropathy in a 35-year-old man. He experienced acute adrenal insufficiency at 29 years of age, followed by psychic disorders and mental deterioration, then spastic paraparesia, dysarthria and peripheral neuropathy. MRI showed high-intensity signal areas in the cerebellum and periventricular white matter. Clinical symptoms, neurophysiological and neuroradiological examinations disclosed both features of adrenoleukodystrophy and adrenomyeloneuropathy. This indicates that adrenoleukodystrophy and adrenomyeloneuropathy probably represent two portions of the same entity.