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PubMed2002

[Lhermitte's sign].

Besides Babinski's, Lhermitte's sign is likely the eponym mostly used in the neurological literature. We review here the history of this eponym as well as recent advances on its pathophysiology and treatment. Lhermitte's phenomenon is, on one hand, a symptom as it is spontaneously explained by patients, and, on the other hand, a sign as it may be triggered by flexion of the nape. Initially described after head and cervical spine trauma, firstly by Marie and then by Babinski, it was Jean Lhermitte who recognized on it an etiological specificity, namely, a demyelinating sign of cervical spinal posterior cords. He also made a pathophysiological interpretation of the phenomenon, namely, a stretching of posterior cords during flexion of the neck. All authors agree that this phenomenon is more common in multiple sclerosis, although it has been descibed in many other conditions. The history of how this sign was hatched, whose pathophysiology remains a mystery, is fascinating. And it is fascinating the fact that patients compare the phenomenon with a current, especially if we bear in mind that few people may have suffered an electrocution, mainly in that time when just a few ones could actually enjoy domestic electricity.

Cervical Vertebrae↗
PubMed2005

Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases.

This report concerns a clinicopathological study of three additional patients with corticobasal degeneration (CBD), described here for the first time, and a clinicopathological correlation between pyramidal signs and upper motor neuron involvement, in ten autopsy cases of CBD, including seven cases reported by us previously. We investigated pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and involvement of the primary motor cortex and pyramidal tract, focusing on the astrocytosis of the fifth layer of the primary motor cortex. Pyramidal signs were observed in six (60%) of the ten cases. Hyperreflexia was evident in six patients (60%), with spasticity being observed in three patients (30%). Loss of Betz cells associated with prominent astrocytosis and presence of ballooned neurons in the fifth layer of the primary motor cortex was observed in all ten cases. In all cases, involvement of the pyramidal tract was obvious in the medulla oblongata, without involvement of the pyramidal tract in the midbrain. Constant and severe involvement of the fifth layer of the primary motor cortex, including the Betz cells, has not previously been reported in CBD. We suggest that the pyramidal signs in CBD have been disregarded.

Aged↗
PubMed2002

Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: a clinicopathological study of eight autopsy cases.

We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.

Adult↗
PubMed1977

Adult-onset hereditary ataxia in Scotland.

A systematic search for cases of adult-onset hereditary ataxia was conducted on location in Scotland. The investigation resulted in the discovery of eight pedigrees with 42 patients of whom 16 were alive in 1975. Nine patients were examined by the authors and recent hospital records were available on the remaining seven. The clinical features were quite variable. In declining order of frequency, findings were gait and limb ataxia, dysarthria, hyperreflexia, extrapyramidal motor disturbances, impaired vibratory sense, spasticity, defects of extraocular movements and nystagmus, reflex depression, Babinski signs, impaired joint position sense, muscle weakness, optic atrophy, and mental abnormalities. Foot deformity occurred only once. Inheritance was compatible with autosomal dominant transmission, but complicated by consanguinity in two families. The minimum prevalence was calculated as 0.31/100,000. Autopsy in two members in one family revealed olivopontocerebellar degeneration.

Adult↗
PubMed1984

Painful ataxic hemiparesis.

Right hemiparesis with right-sided pain and ataxia developed in a 68-year-old man. Sensation, neuropsychological function, and somatosensory evoked potentials were normal. Computed tomography showed an isolated fresh infarct in the left part of the thalamus. The pain and ataxic disturbances were related to involvement of the thalamus itself, but the hemiparesis with hyperactive tendon reflexes and Babinski's sign was probably due to associated dysfunction in the adjacent internal capsule from compression or edema. In the available clinicopathological reports of cases with hemiparesis and thalamic infarction, contiguous involvement of the internal capsule or no associated lesion has been reported. Because of the occurrence of pain, which is not present in pontine, mesencephalic, or capsular ataxic hemiparesis, we suggest that the syndrome seen in our patient be called "painful ataxic hemiparesis."

Ataxia↗
PubMed1992

Flexor plantar responses in children with upper motor neuron lesions.

The usual plantar response in upper motor neuron lesions is extensor. As flexor responses were seen in a number of children with these lesions, a formal study was undertaken to assess the plantar reflex in affected children. Fifty-seven children with spastic cerebral palsy were examined. Ten had hemiplegias, so a total of 104 limbs were tested. Eighty-four (80.8%) of these had flexor and 12 (11%) had extensor responses. Half (3.9%) of the remaining eight responses were mute and half were variable. While exceptions to the extensor rule are known, this marked predominance of flexor responses is unusual. This may be the result of damage inflicted on the immature nervous system before full connections and myelination have been completed. Further studies, involving children who have sustained brain damage at different ages, will be needed to elucidate the various mechanisms involved.

Adolescent↗
PubMed1975

Relationships between neurological findings and classroom behavior.

Five hundred seventy-five children from low-income urban neighborhoods who were between 10 and 12 years of age were examined by pediatricians for certain neurological signs. Classroom teachers ranked each child according to types of behavior. Data on neurological signs found in more than 15 children and on types of classroom behavior clinically expected to be related to central nervous system defects were studied statistically. Significant positive associations were found between nystagmus and hyperactivity, mixed dominance and hyperactivity, and mixed dominance and variable day-to-day performance. Errors in moving parts of the body on verbal command were associated with distractibility and underachievement. Head circumference greater than the 90th percentile for age was associated with unvarying behavior and clumsiness; tactile agnosia with unvarying behavior; asymmetry of the eyes with hyperactivity; and asymmetrical position of the child's head with underachievement. A negative association was found between nystagmus and musical ability.

Agnosia↗
PubMed1976

[Neurological findings (especially "developmental reflexes") in residents of homes for the aged (author's transl)].

86 residents of several homes for the aged in Berlin, aged 75 to 96 years (mean age 86.3 years), were examined neurologically. Many showed the following aberrant neurological signs: Pallaesthesia and dermolexia were extinct in the lower extremities; the ankle jerks could not be elicited; the palmomental, orbicularis oris reflex, grasping and the snout reflexes were positive; there was a hypokinetic-hypertonic motor syndrome. The statistical analyses and the follow-up dynamics of our findings argue for such age-dependent changes of the CNS as the basis for the grasping and snout reflexes and for the involuntary movements of the elderly.

Aged↗
PubMed1986

Long latency muscle responses in cerebellar diseases.

Long latency reflexes were measured from the hand muscles of 27 patients suffering from different cerebellar diseases (12 diffuse cerebellar atrophies, 7 cerebellar hemispheric infarcts, 8 Friedreich's disease) and from 45 controls after electrical stimulus of the median nerve at the wrist. The M3 response (latency about 70 ms) was increased in about 50% of cerebellar atrophy cases and occasionally (10 of 12 cases) separated from the M2 response (50 ms). M3 was sometimes (3/7) increased and the M2-3 complex was prolonged ipsilaterally in cases of cerebellar infarcts. In the cases of Friedreich's ataxia M2 was always lost uni or bilaterally because of the disturbance of afferent or efferent fibres. The latencies of the spinal reflex M1 and also of M2 were not always increased strongly enough to be clearly separated from the normal values.

Adult↗
PubMed2001

Diminished plantar grasp response as an additional indicator of a shunt malfunction in a case of congenital hydrocephalus.

A male infant with congenital hydrocephalus who had undergone ventriculoperitoneal (VP) shunting at 3 days of life exhibited a diminished plantar grasp response (PGR) from 2 months of age, which had clearly recovered 3 months after a shunt revision at 6 months of age. The diminished PGR was the only overt neurological sign in this patient. The precipitous decrease in PGR that occurs during early infancy in a hydrocephalic infant with VP shunting is a possible indicator of a prespastic condition caused by a shunt malfunction.

Equipment Failure↗
PubMed2004

An immunohistochemical study of extracellular matrix proteins laminin, fibronectin and type IV collagen in paediatric glioblastoma multiforme.

AIMS: In the recent decades many studies have been addressed in the literature to assess specific factors related to glioblastoma multiforme (GBM) invasion. However, few studies have evaluated tumour cell's interaction with specific extracellular matrix (ECM) components, and, moreover, there is a lack of information regarding the occurrence of these phenomena in paediatric GBM. METHODS AND RESULTS: ECM proteins were evaluated in six cases of paediatric GBM assessing the immunohistochemical expression of laminin, fibronectin, and type IV collagen. We used a semiquantitative scale, ranging from not detected (zero) to marked (3). Laminin expression was minimal in three cases, moderate in one case, marked and generalised in one patient and marked and focal in the last case. Fibronectin expression was minimal in three patients; moderate immunoreactivity was documented in one case. Conversely, one case was classified as marked with generalised distribution and the remaining case as marked with focal immunostaining. Type IV collagen expression was minimal in three cases, moderate in one, marked with focal reaction in one and marked with generalised reaction in the remaining case. CONCLUSIONS: This study provides additional insights into tumour invasion features of paediatric GBM, as ECM plays a pivotal role in numerous cellular functions during normal and pathological processes. Although based on a limited number of patients, this investigation may serve as a challenge for the management of paediatric GBM, stimulating trials with larger patient numbers aimed at documenting specific factors influencing GBM prognosis.

Biomarkers, Tumor↗
PubMed2005

Acute spinal cord compression in hereditary multiple exostoses.

Osteocartilaginous exostoses are benign bone tumors frequently found in the metaphysis of long bones but rarely in the spine. Four patients with acute spinal cord decompensation due to vertebral exostoses spinal cord compression have been previously described in the literature. We report an additional case of rapidly evolving spinal cord compression due to a cervical osteochondroma in a patient with hereditary multiple exostoses (HME), also known as Bessel Hagen disease. Careful analysis of the 5 cases suggested to us that patients with HME should have a systematic spinal imaging screening, in order to prevent rapid neurological decompensation. A minimal risk surgical procedure can be performed at a time of election.

Adult↗
PubMedDate not supplied

Spatial factors and muscle spindle input influence the generation of neuromuscular responses to stimulation of the human foot.

Removal of the mechanical pressure gradient on the soles leads to physiological adaptations that ultimately result in neuromotor degradation during spaceflight. We propose that mechanical stimulation of the soles serves to partially restore the afference associated with bipedal loading and assists in attenuating the negative neuromotor consequences of spaceflight. A dynamic foot stimulus device was used to stimulate the soles in a variety of conditions with different stimulation locations, stimulation patterns and muscle spindle input. Surface electromyography revealed the lateral side of the sole elicited the greatest neuromuscular response in ankle musculature, followed by the medial side, then the heel. These responses were modified by preceding stimulation. Neuromuscular responses were also influenced by the level of muscle spindle input. These results provide important information that can be used to guide the development of a "passive" countermeasure that relies on sole stimulation and can supplement existing exercise protocols during spaceflight.

Adolescent↗