Median nerve somatosensory evoked potentials recorded with cephalic and noncephalic references in central and peripheral nervous system lesions.

Somatosensory evoked potentials (SSEP) to electrical stimulation of the median nerve by using cephalic and noncephalic references were studied to detect the generator sources of short latency evoked potentials in 29 patients with cerebral, brainstem, spinal and peripheral nerve lesions. Patients were divided into six groups according to the localization of their lesions: group 1: cortical and subcortical lesions, group 2: basal ganglion lesions, group 3: pons and mesencephalon lesions, group 4: diffuse cerebral lesions, group 5: cervical cord lesions, group 6: brachial plexus lesions. Potentials were recorded using cephalic and noncephalic references after median nerve stimulation. Evidence obtained from patients suggested the following origins for these short latency SSEPs: P9 may arise in brachial plexus, P11 in dorsal basal ganglions or dorsal column, P13 and P14 in the nucleus cuneatus and lemniscal pathways, N16 in subthalamic structures and most likely mid and lower pons, N18 from the thalamus and thalamocortical tract, and N20 from primary somatosensory cortex.

Essential startle disease may not be a uniform entity.

A 25-year-old man with essential startle disease has been reported. He had a history of sudden jerks and falls in response to unexpected stimuli. Abnormal falling developed when he learned to walk. No similar cases are known in his family. Physical examination revealed hyperreflexia. Pathologic startle reflex was elicited by light touching on the patient's nose, clapping or making other noises. EEG response to startle stimuli consisted of spikes recorded from both centroparietooccipital regions immediately preceding diffuse muscle and movement artifacts. The motor responses to auditory stimuli, which are startle reflex on the orbicularis oculi, sternocleidomastoid, biceps and quadriceps femoris muscles, habituated within 2-4 trials. Electrical stimulation of the median nerve at the wrist elicited a consistent C reflex (cortical long loop reflex) but not any giant cortical somatosensory evoked potentials. Our patient showed early disease onset and habituation of motor response, which are not seen in other essential hyperekplexia cases, and clinically differs from the patients with hereditary hyperekplexia in which neonatal rigidity, epilepsy, apneic attacks, low intelligence, congenital dislocated hips and inguinal hernia can be seen in differing frequency.

Alpha-interferon and isoprinosine in adult-onset subacute sclerosing panencephalitis.

We report eight patients with adult-onset subacute sclerosing panencephalitis (SSPE), of which, four were treated with oral isoprinosine and four with intraventricular alpha-interferon plus oral isoprinosine. One of the four patients treated with oral isoprinosine died within two months, and the disease progressed in three patients. Of the four patients treated with oral isoprinosine plus intraventricular alpha-interferon, one showed mild progression, one remission, and the remaining two showed stabilization. The group of patients is relatively small, but our results suggest that treatment with oral isoprinosine plus alpha-interferon is effective for SSPE.

Migrainous stroke causing bilateral anterior cerebral artery territory infarction.

A 38-year-old man developed bilateral anterior cerebral artery territory infarction during the course of a migraine. Magnetic resonance imaging showed bilateral ischemic lesions involving the cortex of the paramedian region of the frontal and parietal lobes, more prominent on the right. Cerebral angiography was normal. To our knowledge, this is the first report of bilateral anterior cerebral artery territory infarction from migraine.

Mirror movements associated with cervical meningocele: case report.

A patient with mirror movements associated with cervical meningocele has been presented in this report. The MRI showed normal cerebrum and cervical meningocele, and an anomaly at the posterior to the cervical spinal cord-medulla junction. Unilateral transcranial magnetic stimulation evoked bilateral responses at similar latencies on the thenar muscles which are quite different from those observed in normal subjects. This case adds another etiological cause to the mirror movements.

Radiological follow-up in encapsulated intracerebral hematoma mimicking intratumoural bleeding.

Chronic encapsulated intracerebral hematoma, which is usually seen in young, normotensive patients, is rare, but has been reported with increasing frequency in recent years. In this report, we have presented a case of encapsulated intracerebral hematoma mimicking intratumoural bleeding with its whole natural radiological progression. A 55 year-old man developed a progressive neurological deficit one month after hospitalisation due to spontaneous intracerebral hemorrhage. Cranial CT and MR demonstrated a ring-shaped hemorrhagic lesion with mass effect and perifocal edema. After 15 months, there was marked improvement in clinical findings, and imaging techniques showed marked resorption of the mass. Radiological findings of spontaneous resolution of the encapsulated intracerebral hematoma are described for the first time in the reported case. Encapsulated intracerebral hematoma can present much like a brain tumour and should be considered in the differential diagnosis of other hemorrhagic space-occupying lesions.

Cerebral venous thrombosis in pregnancy: the role of protein S deficiency.

We report a 20 year-old, 28 week-pregnant woman, who developed superior sagittal sinus thrombosis, associated with a decreased free protein S level. Her father, who had been a stroke victim, had also significantly lower level of protein S. In very rare cases, a congenital or acquired protein S deficiency leads to cerebral venous thrombosis. The detection of such abnormalities has major practical consequences for the long-term management of patients to prevent further thrombotic episodes.

Traumatic basilar artery dissection causing locked-in syndrome.

We report on a patient with traumatic dissection limited to the basilar artery causing a locked-in syndrome. Magnetic resonance imaging (MRI) showed infarction of the basis pontis and a high signal intensity ring surrounding the central lumen of the basilar artery. Vertebral angiography revealed a double lumen within the basilar artery, consistent with a dissection. MRI and angiography are complementary to each other, and we consider both to be necessary for accurate diagnosis. Because MRI is less invasive than angiography, MR examinations should be performed before angiography for screening.

Acute fulminant SSPE: clinical and EEG features.

We report an autopsy-proven case of SSPE in which the course was rapidly progressive, leading to death in 2 months. The patient was admitted for investigation of visual symptoms. His examination revealed complete right homonymous hemianopia and myoclonic jerks in the right half of the body. EEG showed high voltage periodic slow-wave discharges that were often associated with jerks. Measles antibody was positive in serum and CSF. MRI showed hyperintensity in the white matter of the occipital poles. The patient became completely blind and had jerks in all four limbs. His condition continued to deteriorate and within 3 weeks he reached a state of deep coma. The myoclonic jerks became less marked and gradually completely ceased. The EEG showed diffuse slow activity without periodic complexes. The patient died approximately 2 months from onset of disease. Brain autopsy revealed severe neuronal loss with abundant inclusion bodies in the remaining neuronal and oligodendroglial nuclei, which was compatible with SSPE.

The value of MRI in a case of Tolosa-Hunt syndrome.

We report a case of Tolosa-Hunt syndrome (THS) in which the lesion has been demonstrated by magnetic resonance imaging (MRI), computed tomography (CT), and angiography. A healthy 23-year-old man developed an acute painful ophthalmoplegia on the right side. CT and MRI scans revealed asymmetric enlargement of the right cavernous sinus with contrast enhancement extending down to the region of trigeminal ganglion. MRI further delineated the detailed anatomical structures of the region and excluded any infiltration of the surrounding tissues by a mass lesion. Cerebral angiography showed a significant decrease in the calibration of petrous segment and a mild decrease in the calibration of cavernous segment of the ipsilateral internal carotid artery. The patient was treated with oral prednisone, 100 mg daily. Neurological findings totally subsided after 2 weeks on corticosteroid and MRI showed resolution of the lesion in the cavernous sinus. The patient was symptom-free for 6 months after discharge. Our findings have suggested that MRI is the most valuable imaging technique for demonstration and follow-up of lesions in the cavernous sinus that are directly responsible for the symptoms of THS and the lesions can be more extensive than was currently believed.

SPECT and MRI findings in a case of extensive neuronal migration disorder.

We report a 20-year-old male with epilepsy, mild mental retardation, growth asymmetry, and MRI and SPECT features of unilateral subcortical ectopic cortex. The neurological examination showed mild growth asymmetry, hemiparesis and hemihypoesthesia and pyramidal signs on the left side. EEG showed focal abnormality in the right frontotemporal region. MRI revealed pachygyria and severe heterotopia associated with some abnormalities of ventricles and cerebellum on the right. Cortical responses were absent on stimulation of the left median and tibial nerves. Central motor conduction time from cortex to left upper extremity was prolonged in magnetic stimulation test. SPECT using 99 mTc-HMPAO revealed increased perfusion of the right subcortical region as compared with those of overlying cortical mantle and opposite hemisphere. To our knowledge, there has been no report documenting such a large and extensive subcortical ectopic cortex which appears as a mass distorting and shifting the middle structure in an adult, such as in our case.