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Tone and reflex development before term.

The evolution of tone and reflexes from 25 weeks postmenstrual age (gestational age plus chronologic age) to term in a population of 42 surviving infants is described. The infants were born in 1983 at the Johns Hopkins Hospital, had birth weights less than 1300 g, were examined weekly until neonatal intensive care unit discharge, and did not develop cerebral palsy. Lower-extremity flexor tone was first detectable at 29 weeks post-menstrual age by the popliteal angle and heel to ear maneuvers. Flexor tone, recoil, and hyperreflexia were all noted 2 to 3 weeks earlier in the lower extremities (33 to 35 weeks) than in the upper extremities (35 to 37 weeks). Hip tone (35 to 37 weeks) followed knee flexor tone, but preceded shoulder tone (37 to 38 weeks). Trunk tone on ventral suspension emerged closer to term (36 to 40 weeks), and more than half of infants evaluated at term continued to demonstrate head lag when pulled to sitting position. The emergence of the primitive and pathologic reflexes reflects (both in timing and pattern) the evolution of tone: development of the reflexes in the lower extremities precedes that of those in the upper extremities, and development of the distal reflexes precedes that of the proximal. Maturation of tone, deep tendon reflexes, pathologic reflexes, and primitive reflexes occurs in an orderly, sequential manner, with a well-defined pattern: caudocephalad (lower extremities to upper extremities) and centripetal (distal to proximal).

Child Development↗

Allan-Herndon syndrome. I. Clinical studies.

A large family with X-linked mental retardation, originally reported in 1944 by Allan, Herndon, and Dudley, has been reinvestigated. Twenty-nine males have been affected in seven generations. Clinical features include severe mental retardation, dysarthria, ataxia, athetoid movements, muscle hypoplasia, and spastic paraplegia with hyperreflexia, clonus, and Babinski reflexes. The facies appear elongated with normal head circumference, bitemporal narrowing, and large, simple ears. Contractures develop at both small and large joint. Statural growth is normal and macroorchidism does not occur. Longevity is not impaired. High-resolution chromosomes, serum creatine kinase, and amino acids are normal. This condition, termed the Allan-Herndon syndrome, appears distinct from other X-linked disorders having mental retardation, muscle hypoplasia, and spastic paraplegia.

Abnormalities, Multiple↗

[A case of orthostatic tremor in parkinsonism].

A 79-year-old woman presenting with orthostatic tremor (OT) was reported. In addition to OT, neurological examination showed mild dementia, bradykinesia, rigidity of the neck and the upper limbs and positive Babinski reflex on the left. These clinical signs and CT as well as MRI findings suggested vascular parkinsonism as its pathological background. Upon standing with her feet together, she rapidly developed rhythmic repetitive contraction of all leg muscles. The shaking disappeared by walking, sitting, or lying down. The EMG recording revealed 4-Hz tremor which consisted of alternating contraction of anti-gravity muscles and their antagonists. The EMG bursts associated with the tremor were synchronous in corresponding muscles of both legs. OT could be bilaterally reset by unilateral voluntary or passive movement of leg. In the supine position, the tremor was not evoked by voluntary contraction of leg muscles against resistance. As the tremor was aggravated by the administration of haloperidol was suppressed by L-DOPA, it was thought to have the pharmacological basis common to the resting tremor of parkinsonism. Furthermore, we postulated that the postural tonus-regulating system, which is thought to set and maintain the tonus of antigravity muscles for standing upright, might be involved in the generation of the rhythmic discharge pattern (reciprocal bursts in a given leg and synchronized bursts in both legs) of OT.

Aged↗

[Diagnostic value of electromyography in revealing the laterality of the motor deficit in brain tumors].

Electromyographic studies of anterior tibial muscles supplemented by simultaneous induction of Babinski's reflex indicated the appearance of bioelectrical activity in the extremities contralateral to the tumour which suggested the generalization of tone alterations and helped determine the laterization of the pathological process even when there were no clinical manifestations of motor deficit. Having analyzed their own and literature data, the authors came to the conclusion that the contralateral response observed on the electromyogram in the form of prolonged bioelectrical activity with simultaneous appearance of tonic tension in the crus flexors in the absence of clinically evident motor deficit is explained by the presence of the crossover protective reflex on the spinal level.

Adult↗

Clinical aspects of La Crosse encephalitis: neurological and psychological sequelae.

Only rarely will a child continue to have focal neurologic findings, such as paresis, for as long as 8 years after LAC encephalitis. Most of the other focal neurologic findings, such as Babinski reflexes, pathologic reflexes, aphasias, choreas, dysarthrias and ataxias, resolve completely. Abnormal electroencephalographic findings during the acute period were note in 86-100% of the cases. On 1-8 year follow-up, EEG abnormalities were noted in approximately 33% of the subjects tested. Seizures are present at high frequency during acute illness and recurrent seizures may occur in 6-13% of the cases 1-8 years after infection. The cognitive and intellectual functioning of children following LAC encephalitis is not significantly different from that of the normal population. As a group, LAC encephalitis victims also function in the normal range in terms of their academic performances. There are individuals, however, who will have suffered permanent destructive lesions resulting in lowered IQ and lowered school performance. Behavioral abnormalities are difficult to measure and when present, difficult to ascribe to a definitive cause.

Adolescent↗

[A case of arterio-venous malformation associated with agenesis of unilateral internal carotid artery (author's transl)].

A case of arterio-venous malformation (AVM) at the right parietal area in association with agenesis of the left internal carotid artery (ICA) is described. A 27-year-old male complained of severe headache and vomiting on September 27th in 1980. He lost his consciousness and became stuporous within several minutes after the ictus. He was then transferred to Kurume University Hospital. On admission, he was semicomatose, anisocoria and left hemiparesis marked in the lower limbs, and bilateral Babinski reflex were noted. A emergency CT scan revealed a round high density area suggesting intracerebral hematoma at the right parieto-occipital area. Right retrograde brachial angiography was then performed which showed a small AVM at the right parietal lobe mainly fed by the right posterior parietal arterial branch, and drained into the superior sagittal sinus, via the subcortial veins. Left MCA bilateral PCA and SCA also were demonstrated on the angiogram. The angiogram suggested on abnormality of the Willis ring. Emergency operation fro the small AVM associated with intracerebral hematoma was then performed. The AVM was sufficiently removed and approximately 80 gr. of intracerebral clots were also evacuated completely. Histological examination indicated a typical small AVM. The postoperative course was uneventful. Left CAG performed by seldinger method from the femoral artery after the operation showed no demonstration of the left ICA. the left common carotid artery was terminated as the external carotid artery without carotid bifurcation at the neck. The left ophthalmic artery was fed by the meningeal artery of the internal maxillary artery. Left vertebrobasilar system was normal. From an angiogram, the agenesis of the left ICA was most suspected. An agenesis of ICA was uncommon among the literature in which there was no case with this anomaly associated with cerebral AVM. The embryological consideration about this case was mainly discussed.

Adult↗

[Linkage study of hereditary spinocerebellar ataxia, and probable correlation for the loci to the disease phenotypes].

To study gene loci and disease phenotypes, 18 families with dominant OPCA were subjected for linkage analysis to SCA1- or SCA2-linked microsatellites. Total individuals consisted of 190. Among them, 77 were affected. Consequently, 10 families were 6p-linked, 7 were 12q-linked, and one was type-undetermined. These results indicate that the majority of dominant OPCA in Japan are composed with these two genotypes. Clinically, these two disorders show progressive ataxia, Babinski reflexes, and terminal amyotrophy. Other common features in SCA1 were hyperreflexia, spasticity, mild nystagmus at early stage, slow saccade, and external ophthalmoparesis (EOP) at advanced stage. In contrast SCA2 showed progressive hyporeflexia and slow saccade from early stage. Moreover, choreiform movement, tremor, and rhythmic myoclonus were more frequent in the latter. Neuropathologically, dentate nucleus, brainstem motor nuclei, spinocerebellar tract were involved more severely in SCA1 than SCA2. Degeneration of substantia nigra is more marked in SCA2 than SCA1. These observations strongly indicate that there are correlations between genotypes and phenotypes in dominant OPCAs. Conversely, it is possible to diagnose these two genetic disorders from the clinico-pathological findings.

Chromosome Mapping↗

The optometric assessment of neurologic function.

BACKGROUND: As a direct extension of the nervous system, the eye may be involved in a number of nervous system diseases and disorders. METHODS: The previous ophthalmic and medical literature was evaluated and organized into an orderly review of the literature with recommendations for the examination of patients with neurologic dysfunctions. RESULTS: Many of the appropriate tests for the assessment of neurologic function can be readily performed by optometrists in the clinical setting. CONCLUSIONS. The screening of neurologic function by optometrists is important in many ways. The optometrist can gain significant insight in the ocular and systemic diagnoses associated with neurologic disease by utilizing the appropriate clinical tests and as a result then communicate more effectively with neurologists and other health care practitioners.

Blinking↗

[A case of paradoxical brain embolism in a 17-year-old boy].

A 17-year-old healthy high school boy suddenly suffered from consciousness disturbance immediately after a karate match. His neurological findings on admission were consciousness disturbance, motor aphasia, right hemiparesis, and right Babinski reflex. Cerebral angiography showed occlusion of the precentral branch of the left middle cerebral artery, and X-ray CT scan on the second day revealed low density area in its territory. No abnormality was found on transthoracic echocardiography or Holter ECG. Contrast transesophageal echocardiography with Valsalva maneuver was performed twice (the 2nd and the 14th day), and patent foramen ovale (PFO) was found only on the second trial. It was supposed that paradoxical embolism had occurred. Transesophageal echocardiography is necessary for the embolic patients of unknown origin, especially young adults. To find PFO, it is important to repeat contrast transesophageal echocardiography carefully. if it is negative on the first trial.

Adolescent↗

[Babinski sign].

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Humans↗

[A case of spinal epidural hematoma associated with idiopathic thrombocytopenic purpura].

Spinal epidural hematoma is a rare clinical entity, and the literature provides reports of 29 cases so far in Japan. A case of spinal epidural hematoma associated with idiopathic thrombocytopenic purpura diagnosed by CT scan and MRI is reported in detail with references to the literature. A 56-year-old female was admitted to our hospital on April 22, 1990, because of sudden onset of nuchal pain and right hemiparesis. Her consciousness was alert, but the deep tendon reflex was depressed, and pathological reflex such as Babinski's reflex was positive on the right side. Nuchal stiffness was observed. CT scan of the head revealed no abnormality, but the scan of cervical area showed an abnormal high density area in the right posterior region of the spinal cord at C2 - 3 level. MRI also revealed a low intensity area in the same region both in T1 and T2 weighted images. On admission, the platelet count was 10,000/microliters, and the bone marrow aspirate showed abundant megakaryocytes. The patient was diagnosed as having spinal epidural hematoma associated with idiopathic thrombocytopenic purpura. The patient was initially treated with a corticosteroid and a hyperosmotic agent. About 15 hours after the onset, her motor function began improving. Conservative therapy was continued, and she could walk 2 weeks after the onset. Spinal epidural hematoma is an uncommon disease commencing with back and radicular pain, paraplegia and rectovesical insufficiency. Early diagnosis and surgical decompression is generally imperative, although an exceptional remission without operation such as was observed in this case may occur.

Female↗