1/13. The significance of a crossed extensor hallucis response in neurologic disorders: a comparison with the Babinski sign. The significance of a crossed extensor hallucis response on active flexion of the hip was analysed in various neurological disorders. It is concluded that this sign, not formerly described, is a pathological reflex or synkinesia. In the cooperative patient the crossed extensor response is a more sensitive indicator of a minor disturbance within the cortico-spinal motor pathways than the Babinski sign. With lesions above the foramen magnum a crossed extensor hallucis response is observed more frequently than the Babinski sign. ( info) |
2/13. 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. ( info) |
3/13. 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. ( info) |
4/13. Phenotypic presentation of frontotemporal dementia with Parkinsonism-chromosome 17 type P301S in a patient of Jewish-Algerian origin. A 39-year-old old Jewish woman of Algerian origin developed a rapidly progressive neurocognitive disorder characterized by asymmetric rigidity, spasticity with bilateral Babinski's sign, bradykinesia, altered speech that progressed to mutism, and severe bradyphrenia. She partially responded to levodopa. The family history revealed 4 affected first-degree relatives (3 had already died). Genetic studies carried out in the proband and her living affected sister showed a P301S mutation in chromosome 17. ( info) |
5/13. Babinski-Nageotte's syndrome and Hemimedullary (Reinhold's) syndrome are clinically and morphologically distinct conditions. A hemimedullary infarction, in which medial and lateral medullary lesions occur simultaneously, is a rare cerebrovascular disease. It has been suggested that the Babinski-Nageotte's syndrome is the classical brainstem syndrome that corresponds to hemimedullary lesion. In this study we compare clinical symptoms and magnetic resonance imaging (MRI) data of two patients exhibiting classical Babinski-Nageotte's syndrome according to the original description with symptoms and MRI data of a patient with clinically complete hemimedullary lesion. Our study shows that Babinski-Nageotte's syndrome is neither clinically nor on MRI identical with hemimedullary lesion. Hypoglossal palsy, an invariable symptom of hemimedullary syndrome, is not part of the Babinski-Nageotte's syndrome according to the original description. Consistent with the original historical report, Babinski- Nageotte's syndrome is a lateral "Wallenbergian" medullary lesion with a spreading of the lesion to the more basal localised pyramidal tract. The clinical features of the hemimedullary syndrome, described in 1894 by Reinhold, and the MRI appearances in our patient with this syndrome are clearly different from other classical brainstem syndromes and should be called Reinhold's syndrome. ( info) |
6/13. Hereditary neuropathy with liability to pressure palsies (HNPP) in a toddler presenting with toe-walking, pain and stiffness. The typical clinical presentation of hereditary neuropathy with liability to pressure palsies is an adult-onset recurrent, painless monoparesis. Electrophysiological abnormalities--decreased nerve conduction velocities and delayed distal latencies--can be detected even in asymptomatic patients. We describe a toddler, who presented with asymmetric toe walking, painful cramps and stiffness in the legs. He had calf hypertrophy, brisk tendon reflexes and bilateral Babinski signs and the electrophysiological examination was normal. The unlikely diagnosis of hereditary neuropathy with liability to pressure palsies was reached 5 years later, when the boy started to complain of episodic numbness and weakness in the upper extremities. His father, paternal aunt and grandmother had similar symptoms, but they had never been investigated. The typical 1.5 Mb deletion on chromosome 17p11.2-12 was found in our patient and his affected relatives. ( info) |
7/13. HTLV-I associated myelopathy/tropical spastic paraparesis in a 7-year-old boy associated with infective dermatitis. HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic myelopathy characterized by a slowly progressive spastic paraparesis and sphincter disturbances beginning in adulthood. Only eight well-documented cases occurring in childhood and adolescence have been described. Infective dermatitis associated to the HTLV-I (IDH) is a chronic eczema of childhood occurring in vertically infected carriers. Here we describe a 7-year-old boy with HAM/TSP and IDH. The neurological manifestations were spastic gait, hyperreflexia of lower limbs, clonus and bilateral Babinski's sign. High levels of htlv-i antibodies in the serum and in the cerebrospinal fluid were observed. The association of these two diseases and the early onset of HAM/TSP are probably related to a strong humoral anti-HTLV-I response. ( info) |
| 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. ( info) |
9/13. The Babinski sign and the pyramidal syndrome. The presence or absence of a Babinski sign can be puzzling, but in the light of existing pathological studies it is more fruitful to consider which pyramidal tract fibres release it than whether they release it. This was investigated clinically, by looking for correlations with other reflex changes and with motor deficits in the leg. A survey of 50 patients with a unilateral Babinski sign and six patients who lacked it in spite of other pyramidal tract signs was supplemented with follow-up of the patients who had acute lesions. Appearance of the Bibinski sign proved to depend on the interaction of two factors: (1) activity (not necessarily hyperactivity) in the segmental pathways of the flexion synergy; (2) a motor deficit of the foot, in some cases consisting only in an impairment of rapid foot movements, and probably representing a disturbance of direct pyramidal tract projections to distal motoneurones. ( info) |
10/13. Persistent neurological deficit precipitated by hot bath test in multiple sclerosis. For a half century, the hot bath test has been used as a "diagnostic test" in multiple sclerosis. The appearance of new neurological signs or aggravation of preexisting signs generally is transient, with resolution on return of body temperature to normal. We have observed four patients, however, with considerable and prolonged neurological debilitation after hot bath testing. We suggest caution in the application of such testing. ( info) |