Melanocytic meningitis and large congenital melanocytic naevus: neurocutaneous melanosis

Neurological symptoms in a patient with large congenital melanocytic naevus are highly suggestive of cerebromeningeal melanoma metastasis. The presence of melanocytic cells in cerebrospinal fluid confirms this diagnosis If their malignant nature is shared with cutaneous naevocytic cells. Conversely, neurocutaneous melanosis is diagnosed when benign melanocytosis meningitis is found in patients with multiple and/or large congenital melanocytic naevus, whether cutaneous naevus cells are benign or not, or when cerebrospinal fluid cells are malignant with benign cutaneous melanocytic naevus.We report the case of a young man aged 19 presenting with multiple and large congenital melanocytic naevus who experienced transcient neurological signs and increased intracranial pressure. Cerebral neuroimaging evoked meningeal infiltration which benign melanocytic nature was supposed on CSF analysis and confirmed by necropsy findings, only 3 month after neurological onset, leading to neurocutaneous melanosis diagnosis. This rare neuroectodermal dysembryoplasia finds expression in various neurological signs, depending on patient's age and leptomeningeal and/or cerebral proliferation localization. Lumbar puncture, cerebral scanography and MRI may help diagnosis, but only histological examination can prove neurocutaneous melanosis, more often by necropsy because of poor prognosis.     

Linear cutaneous lesions and neurological deficit. A case report

A patient with subdural hygroma in the perinatal period developed into a right hand sided spastic hemiplegia with epileptic seizures and intellectual deficit. On the age of 17 years a linear dermatosis that proved to be a naevus unislateris was remarked. The combination of linear nevi and neurological deficit is known as the epidermal nevus syndrome.     

Neurocutaneous melanosis, neurofibromatosis and spinal meningioma: an unusual association

The case of 38-year-old woman bearer of a congenital giant naevus "en pélerine" with numerous neurofibromas and other satellite naevi was reported: the patient was afflicted by spastic tetraparesis, more pronounced on the right side. MRIscan of the spine revealed the presence of a cervical spinal tumor shown histologically to be a psammomatous meningioma. The skin picture was consistent with neurocutaneous melanosis; the rarity of its association with neurofibromatosis and spinal meningioma is discussed in the light of embryologic arguments.     

Basal cell naevus syndrome and intracranial meningioma.

Two cases of the basal cell naevus syndrome in association with an intracranial meningioma are described and the relationship discussed.     

A neuropathological study of two autopsy cases of syndromic hemimegalencephaly

Hemimegalencephaly (HMEG) is a malformation of cortical development characterized by unilateral enlargement of the cerebral hemisphere, severe architectural and cellular abnormalities and association with intractable epilepsy. HMEG may represent an isolated lesion of the central nervous system, but may also be associated with several neurocutaneous syndromes. In the present study we discuss the neuropathological findings of two autopsy cases of HMEG associated with linear naevus sebaceous syndrome. Both cases showed the presence of linear naevus sebaceous on extensive areas of the face. The neurochemical profile of the glial and neuronal components in the affected hemisphere was determined using immunocytochemical markers and was compared with the unaffected contralateral hemisphere and normal control tissue. The observed cytomegalic neurones expressed receptors for distinct neurotransmitters, neuropeptides and growth factors. Analysis of components of the phosphoinositide 3-kinase pathway revealed expression of phospho-S6 ribosomal protein in cytomegalic neurones. Autopsy findings confirm the complexity of the histologic phenotypic manifestations in HMEG and proved useful in determining the spectrum of cytoarchitectural and neurochemical abnormalities, underlying the molecular pathogenesis and epileptogenesis of this brain malformation.     

HEMIMEGALENCEPHALY: A CLINICOPATHOLOGICAL STUDY OF FOUR CASES

Pathological findings in four cases of hemimegalencephaly are presented. These cases demonstrated diffuse enlargement of the cortex with disappearance of horizontal layering of the neurons restricted to one hemisphere. In all cases there was marked enlargement of a high percentage of neurons. The nosological situation is discussed and is considered to be heterogeneous; cases 1-3 had cerebral lesions without lesions of the skin or viscera. In these three cases, significant glial abnormalities were found in only one. These cases and others quoted in the literature were considered to be a separate entity completely different from Bourneville's tuberous sclerosis. The aetiology of this developmental malformation remains unknown; genetic factors were not found. Case 4 was associated with a cardiac lesion and with a linear sebaceous naevus characteristic of Solomon's syndrome. Periventricular tumours resembling candlewax drippings were noted. This case, close to Bourneville's disease, could be incorporated into the framework of neuroectodermoses together with tuberous sclerosis.     

Neurocutaneous melanosis

The authors report on a patient of 33 years of age suffering from neurocutaneous melanosis in whom two years after excision of a naevus pigmentosus et pilosus giganteus cellular proliferation of the melanoblasts in the area of the soft meninges was seen. This process led to the death of the patient within a few weeks' time despite intensive therapeutic measures, the manifestation being that of a generalised space-occupying growth in the CNS. Neuroradiological techniques can objectify the clinical pattern of signs of this very rare disease and may possibly enable an early diagnosis.     

Early diagnosis of multiple sclerosis

Tuberous sclerosis belongs to the diseases diagnosed usually at pre-school or school age. Pringle's naevus ist the basic sing indicating the diagnosis. Genetic determination of the disease makes possibly early diagnosis necessary for correct genetic counselling. In the light of long-term observations of children with tuberous sclerosis the authors discuss the problems connected with early diagnosis of this condition evaluating the diagnostic usefulness of various signs and symptoms of the disease, and the usefulness of radiological examination methods. The importance of computer tomography for early diagnosis, and of family examination in cases of tuberous sclerosis is emphasized.     

Cellular blue naevus of the scalp with brain invasion.

A case of a 28 year old woman with an intracranial cellular blue naevus (CBN) which was believed to be the extension from a pigmented skin/scalp lesion is reported. There was no similar pigmented skin lesion noted on other parts of her body. Radical intervention, including wide excision of the affected skin of the scalp, removal of the underlying pigmented skull bone and wide excision of the pigmented dura, together with wide excision of the intracranial mass, were performed. The skin defect was covered by rotation flap and free skin graft. The dura was closed by grafting with fascia lata. The skull defect was left open and would be repaired later at a second planned surgery.     

The inverse relationship between nonverbal intelligence and the latency of the Hoffmann reflex from the right and left thenar muscles in right- and left-handed subjects

The relationship between nonverbal reasoning ability and the latency of the Hoffmann (H) reflex was studied in right- and left-handed subjects. The nonverbal reasoning ability was assessed by the Cattell's Culture Fair Intelligence test. Hand preference was assessed by the Edinburgh Handedness Inventory. Hoffmann reflex was recorded from thenar muscles of the right and left thumbs. In left-handers (total sample), IQ was found to be significantly and negatively linearly related to the H-reflex latency from the left side. In strong left-handers, there was a significant negative linear relationship between IQ and the H-reflex latency from right and left sides, but with a higher correlation for the left side. In weak left-handers, IQ did not show any significant relation to the H-reflex latencies from the right and left sides. In right-handers (total sample), there was a significant negative linear correlation between IQ and the H-reflex latency from the right side. In right-handed women without familial sinistrality (FS-), IQ was found to be inversely related to the H-reflex latency from the right and left side, but with a higher correlation for the right side. In right-handed women with FS, IQ did not show any significant relation to the H-reflex latency from the right and left sides. In right-handed men with right eye preference and FS-, IQ was found to be negatively linearly related to the H-reflex latency from the right and left sides equally. In right-handed men with right eye FS+, mixed eye FS-, +, mixed eye-right foot (FS-), mixed eye-mixed foot, and left eye preference there was no significant relationship between IQ and the H-reflex latency from the right and left sides. Only in strong left-handers there was a significant, positive linear correlation between IQ and the right minus left (R-L) difference of latencies. The H-reflex latency from the right and left sides was not significantly correlated with the R-L difference of latencies in left-handers. In right-handed women with right eye preference FS-, and in right-handed men with right eye preferences FS-, The H-reflex latency from the right side was found to be positively linearly correlated with the R-L difference of latencies. In right-handed women FS+, and in right-handed men FS+, and mixed or left eye preference, the H-reflex latency from the left side was found to be negatively linearly related to the R-L difference of latencies.(ABSTRACT TRUNCATED AT 400 WORDS)     

Lateral dominance and aesthetic preference

Left and right handers were found to differ in their preferences for mirror versions of vacation slides. Preferences of one group of right handers were predictive of preferences for another group of right handers, but not of left handers. In a second study, it was found that slides strongly prefered by dextrals were those with the more important content, or the greater heaviness on the right, and that slide asymmetry was highly correlated with dextral, but not sinistral preferences. Results were interpreted as reflecting a left field perceptual bias induced by selective right hemisphere activation in right handers.     

The lateralization of the grasp reflex in human newborns.

The strength of the grasp reflex from the right and left hands was estimated in male and female newborns within the second day after birth. In the total sample (N = 121), the mean strength of the grasp reflex from the right hand was found to be significantly higher than that from the left hand. There was a significant positive linear correlation between reflex strengths from the right and left hands. The mean right minus left (R - L) reflex strength was found to be significantly larger than zero. There was a positive linear relationship between the R-L reflex strength and the reflex strengths from the right and left hands. This was more pronounced for the right hand than the left hand. The reflex strengths from the right and left hands were found to be positively linearly correlated with the weight of the newborn babies. These results indicated a grasp reflex lateralization in newborns. The right-bias in the grasp-reflex asymmetry established in this work would create a basis for the development of the right-hand preference in adulthood.     

A case of adult moyamoya disease showing progressive angiopathy on cerebral angiography.

In moyamoya disease, progression of carotid occlusive lesion in an adult patient is very rare. We report a case of adult moyamoya disease with acute angiographical stage progression and hemodynamic deterioration. A 56-year-old female complaining of left motor weakness visited our department. On MRI, infarct lesion was found in the right white matter. On cerebral angiography, occlusive lesions in the bilateral internal carotid arterial siphons and moyamoya vessels were found. The right side was stage V and the left side was stage III. On IMP-SPECT, decreased cerebral hemodynamic reserve of the right cerebral hemisphere was found. In this patient, right STA-MCA anastomosis was performed. After operation, she became possible to walk and discharged to home. Five months after operation, good collateral formation and improvement of hemodynamic reserve in the right hemisphere were found. However, on left carotid arteriography, the anterior and middle cerebral arteries were only slightly imaged, and the disease state progressed to stage IV. In addition, decreased blood flow and hemodynamic reserve were appeared in the left hemisphere.     

Frequency and urgency of micturition in hemiplegic patients: relationship to hemisphere laterality of lesions

Summary To determine the individual contribution of the right and left cerebral hemispheres to micturition control, a clinical analysis was performed concerning frequency and urgency of micturition in 134 chronic hemiplegic patients. A mean frequency of urination, 9 times or more in 24h, was found more frequently in left than right hemiplegics. Left hemiplegics also complained more often of urgency than did right hemiplegics. A mean frequency of urination of 9 times or more in 24h and urgency co-existed more frequently in left hemiplegics than in right hemiplegics. In the present study, dealing with the chronic sequelae of stroke, frequency and urgency of micturition were found more commonly in patients with right hemisphere than left hemisphere lesions.     

Functional asymmetry in the reading of Braille

Difference between right and left handed reading was investigated in two experiments. In the first, summarised previously, children were found to be faster and more accurate when reading with fingers of the left than the right hand. In the second study, using adults, no difference in speed was found, but fewer mistakes were made when reading with the left than with the right middle finger. The findings are discussed in relation to training effects and cortical asymmetry.     

Preferred hand and familial sinistrality in dichotic monitoring

Ear asymmetries in dichotic monitoring performance were assessed in 105 subjects (77M, 28F). A right ear advantage (REA) was found more often in right than mixed or left handed subjects. Handedness did not affect the magnitude of asymmetry. Familial sinistrality could only be examined in male left handers, and was found to increase the incidence of REA's. Non-familial sinistrals were equally distributed in terms of left, right and no ear advantage.     

Dependence of intermanual difference in hand skill on right or left cerebral motor control in right-handed male and female subjects.

The relations of the mean differences in right and left hand skills (L-R) to the mean right and left hand skills were studied in right-handed male and female subjects with and without familial sinistrality (FS+, FS-). Hand skill was measured by the peg moving task. In FS+ males, there was a significant negative linear correlation between L-R and right hand peg moving times (PMTs). In FS+ females, there was a significant positive linear correlation between L-R and left hand PMTs. There were no significant correlations between L-R and left hand PMTs in FS+ males, and right hand PMTs in FS+ females. FS- subjects could be described as follows. In males and females with right eye and right foot preference, L-R PMTs were found to be positively linearly correlated with left hand PMTs (no correlation between L-R and right hand PMTs). In subjects with left eye and right foot preference, the correlations were found to be similar to those for FS+ subjects. In subjects with mixed eye and right foot preference, there was a negative linear correlation between L-R and right hand PMTs in females; a positive linear correlation between L-R and left hand PMTs in males. There were no significant correlations between L-R and right hand PMTs in males, and left hand PMTs in females. These results indicated that left brain in FS+ males, and right brain in FS+ females would produce the L-R difference between hands in PMTs.(ABSTRACT TRUNCATED AT 250 WORDS)     

A comparison of left- and right-arm movements during speaking

Arm movements were both videotyped and recorded with potentiometers attached to the arms, during speaking. Flexion-extension and supination-pronation were compared in detail in left and right arms during free movements made bilaterally or with one arm at a time. The right arm was found to make more direction changes per movement than the left arm, but no differences in onset or termination time, or in duration of movement were found. The results are discussed in terms of the nature of left-hemisphere control of left and right arms in proximal free movement.     

Right and left hand skill in relation to cerebral lateralization in right-handed male and female subjects: the prominent role of the right brain in right-handedness.

The associations between right and left hand skills to cerebral motor lateralization were studied in right-handed subjects with (FS+) and without (FS-) familial sinistrality. Hand preference was assessed by Oldfield questionnaire and hand skill by the peg moving task. The mean peg moving times (PMTs) and their standard deviations (SDs) were calculated for each hand. There was a significant positive linear correlation between the mean right and left hand PMTs. In males, the mean difference between the left and right PMTs (L-R) showed a significant negative linear correlation with the mean right hand PMTs. There was no significant correlation between these parameters in females. The mean L-R PMTs were found to be positively linearly correlated with the mean left hand PMT in FS- males, FS- and FS+ females (no correlation in FS+ males). The results indicated the contribution of the mean left hand PMTs to L-R hand skill is much higher than that of the mean right hand PMTs. In males, there was no significant correlation between SDs of the mean right and left hand PMTs. In females, SD of the mean right hand PMT was found to be positively linearly related to SD of the left hand PMT. In males, L-R SDs were found to be negatively linearly related to SDs of the mean right hand PMTs and positively linearly related to the SDs of the mean left hand PMTs. In females, only the mean left hand PMT positively linearly correlated with L-R SDs. Here again, the left hand prominently influenced the L-R SDs. In males, SD for the mean right hand PMT showed no relation to right hand PMT. There was, however, a negative linear correlation between SD of the mean right hand PMT and the left hand PMT (no correlations in female). It was concluded that the left hand (right brain) would be of higher significance than the right hand (left brain) in determining the degree of the right-bias in hand skill and its stability in right-handers.     

Right fronto-parietal dysfunction in children with ADHD and developmental dyslexia as determined by line bisection judgements

Visual line bisection is a reliable and valid laterality task that is typically used with patients with acquired brain damage to assess right hemisphere functioning. Neurologically normal individuals tend to bisect lines to the left of the objective midline whereas those with right parietal damage bisect lines to the right. In this study children with ADHD were matched with children with developmental dyslexia on IQ and gender to test the hypothesis that right hemisphere neurological abnormalities underlie the behavioural deficits observed in these two disorders. Line bisection performance was compared between groups as a function of response hand, scanning direction and line position. In contrast to results typically found with neurologically normal children, a rightward bias was found for both clinical groups, but to different degrees depending on which hand was used to bisect lines. These findings suggest pathology of the corpus callosum and/or the right fronto-parietal cortex.