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At least 433 records · Page 24Linked to original sources
PubMed2001

Recognizing periodic alternating nystagmus.

Congenital or acquired periodic alternating nystagmus (PAN) is characterized by nystagmus occurring in a cycle. The cycle consists of a left-beating nystagmus, a transition phase, a right-beating nystagmus, and a further transition phase. The purpose of this review is to assist the clinician in the recognition of periodic alternating nystagmus (PAN), either as a type of congenital nystagmus or in its acquired form, and to highlight why such identification is important. Recent studies using eye movement recordings are reviewed to point out the frequency of congenital PAN in samples of patients with congenital nystagmus, and to describe the characteristics of the waveforms and the influence of foveation time on the alternation of head turns. Classical and new surgical alternatives are reported. The identification of congenital PAN is essential when surgical treatment is being considered for the correction of anomalous head postures. Acquired PAN is usually due to cerebellar disease and causes oscillopsia. Unlike other forms of acquired nystagmus, it responds well to drug treatment.

Baclofen↗
PubMed1980

Motor speech disorders: a clinical approach.

Disorders of motor speech may result from impairments in basic motor control systems similar to those causing motor disturbances of the trunk and extremities. Dysarthria and dyspraxia are two distinct disorders of motor speech which differ in both pathologic localization and clinical presentation. These motor speech disorders must be clearly differentiated from other linguistic disorders which result from brain damage. A clear understanding of the relationship among basic neuroanatomy, neurophysiology, neuropathology, and functional clinical assessment will provide the clinician with valuable diagnostic information. This should lead to early referral to the speech pathologist for appropriate management.

Basal Ganglia Diseases↗
PubMed1996

Cerebellar mutism following posterior fossa tumour surgery.

Two cases of transient mutism following operative removal of cerebellar medulloblastoma are reported. These add to the few cases reported in the literature of this rare complication of posterior fossa tumour surgery in children.

Articulation Disorders↗
PubMed2004

An animal model for the tickborne flavivirus--Omsk hemorrhagic fever virus.

The tickborne encephalitis (TBE) serocomplex of flaviviruses consists primarily of viruses that cause neurologic disease; these viruses include Omsk hemorrhagic fever virus (OHFV), a virus that is genetically related to other TBE serocomplex viruses but that circulates in an ecologically distinct niche and causes markedly different human disease. The objective of this study was to examine a potential small-animal model for OHFV and to compare the pathology of infection with that of the neurotropic tickborne flavivirus, Powassan virus (POWV). POWV-infected BALB/c mice demonstrated typical arboviral encephalitis, characterized by paresis and paralysis before death, and viral infection of the cerebrum, characterized by inflammation and necrosis. In contrast, lethal OHFV infection did not cause paralysis or significant infection of the cerebrum but showed marked involvement of the cerebellum. Distinct pathological results in the spleens suggest that the immune response in OHFV-infected mice is different from that in POWV-infected mice. This study demonstrates a clear pathological difference between OHFV-infected mice and POWV-infected mice and supports the use of the BALB/c mouse as a disease model for OHFV.

Animals↗
PubMed1979

Pontine and extrapontine myelinolysis.

No coherent theory has been advanced to explain either the particular localization of the myelinolytic lesions of central pontine myelinolysis or their pathogenesis. However, several lines of evidence support the generalization that the centre of the basis pontis has a special susceptibility to a metabolic fault. The constancy of localization of the lesion and its bilateral symmetry are the very attributes that characterize other metabolic (nutritional) disorders, such as the assymmetrical degeneragion of the papillomacular bundles within the optic nerves and tracts in deficiency amblyopia and the specific affection of the paraventricular regions in the Wernicke-Korsakoff syndrome. And clinically, the frequent occurrence of central pontine myelinolysis in a setting of severe metabolic derangement, particularly of the serum sodium, points in the same direction.

Adult↗
PubMed1990

Symptomatic and essential rhythmic palatal myoclonus.

Rhythmic palatal myoclonus (RPM) is a rare movement disorder consisting of continuous synchronous jerks of the soft palate, muscles innervated by other cranial nerves and, rarely, trunk and limb muscles. It usually develops secondary to brainstem or cerebellar disease (symptomatic RPM). Some patients, however, fail to show evidence of a structural lesion (essential RPM). A total of 287 cases with RPM from the literature including 210 cases with symptomatic and 77 cases with essential RPM have been reviewed and analysed statistically to look for criteria separating the two conditions. Patients with essential RPM usually have objective earclicks as their typical complaint which is rare in the symptomatic form. Eye and extremity muscles are never involved. The jerk frequency is lower in essential than in symptomatic RPM. Patients with essential RPM are younger and have a balanced sex distribution as compared with a male preponderance in the symptomatic form. The rhythmicity of RPM seems to be more profoundly influenced by sleep, coma and general anaesthesia in essential than in symptomatic RPM. We conclude from these results that essential RPM should be separated as a distinct clinical entity. Symptomatic RPM is a rhythmic movement disorder whose pathogenesis is quite well established. The cells of the hypertrophied inferior olives are believed to represent the oscillator. Among other possibilities, essential RPM may represent its functional analogue, based on transmitter changes only. Such a relationship could be of theoretical interest for the understanding of rhythmic hyperkinesias in general.

Age Factors↗
PubMed2000

Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population.

Acquired cerebellar lesions in adults have been shown to produce impairments in higher function as exemplified by the cerebellar cognitive affective syndrome. It is not yet known whether similar findings occur in children with acquired cerebellar lesions, and whether developmental factors influence their presentation. In studies to date, survivors of childhood cerebellar tumours who demonstrate long-term deficits in cognitive functions have undergone surgery as well as cranial irradiation or methotrexate treatment. Investigation of the effects of the cerebellar lesion independent of the known deleterious effects of these agents is important for understanding the role of the cerebellum in cognitive and affective development and for informing treatment and rehabilitation strategies. If the cerebellar contribution to cognition and affect is significant, then damage in childhood may influence a wide range of psychological processes, both as an immediate consequence and as these processes fail to develop normally later on. In this study we evaluated neuropsychological data in 19 children who underwent resection of cerebellar tumours but who received neither cranial irradiation nor methotrexate chemotherapy. Impairments were noted in executive function, including planning and sequencing, and in visual-spatial function, expressive language, verbal memory and modulation of affect. These deficits were common and in some cases could be dissociated from motor deficits. Lesions of the vermis in particular were associated with dysregulation of affect. Behavioural deficits were more apparent in older than younger children. These results reveal that clinically relevant neuropsychological changes may occur following cerebellar tumour resection in children. Age at the time of surgery and the site of the cerebellar lesion influence the neurobehavioural outcome. The results of the present study indicate that the cerebellar cognitive affective syndrome is evident in children as well as in adults, and they provide further clinical evidence that the cerebellum is an essential node in the distributed neural circuitry subserving higher-order behaviours.

Adolescent↗
PubMed1976

Disorders in cerebellar ocular motor control. I. Saccadic overshoot dysmetria. An oculographic, control system and clinico-anatomical analysis.

Photoelectric eye movement recording in 9 patients with cerebellar disorders defined three features of saccadic overshoot dysmetria: (i) saccades were hypermetric and successively diminished in amplitude; (ii) saccadic initiation interval averaged 173 ms: and (iii) eye position was constant during the intersaccadic period. These characteristics indicated that the visually evoked saccades subserving foveation had increased gain, and were modelled by computer simulations using a sampled-data control model with increased feed-forward gain. Eight patients with saccadic overshoot dysmetria had cerebellar neoplasms, vermis-splitting surgical procedures and mid-line cerebellar signs. This clinical evidence suggests that vermian dysfunction is responsible for saccadic overshoot dysmetria. Normally, the cerebellar vermis appears to play an adaptive role by continuously adjusting gain of the direct visual motor pathway. When cerebellar disorder exists, adaptive gain modulation is lost, and, if gain then increases, saccadic overshoot dysmetria is a result.

Astrocytoma↗
PubMed1997

Rehabilitation of balance in two patients with cerebellar dysfunction.

The treatment of two patients with cerebellar dysfunction is described. One patient was a 36-year-old woman with a 7-month history of dizziness and unsteadiness following surgical resection of a recurrent pilocystic astrocytoma located in the cerebellar vermis. The other patient was a 48-year-old man with cerebrotendinous xanthomatosis (CTX) and diffuse cerebellar atrophy, and a 10-year history of progressive gait and balance difficulties. Each patient was treated with a 6-week course of physical therapy that emphasized the practice of activities that challenged stability. The patient with the cerebellar tumor resection also performed eye-head coordination exercises. Each patient had weekly therapy and performed selected balance retraining exercises on a daily basis at home. Measurements taken before and after treatment for each patient included self-perception of symptoms, clinical balance tests, and stability during selected standing and gait activities; for the patient with the cerebellar tumor resection, vestibular function tests and posturography were also performed. Both patients reported improvements in symptoms and demonstrated similar improvements on several kinematic indicators of stability during gait. The patient with the cerebellar tumor resection improved on posturography following treatment, whereas the patient with CTX improved on clinical balance tests. This case report describes two individualized treatment programs and documents functional improvements in two patients with different etiologies, durations, and clinical presentations of cerebellar dysfunction. The outcomes suggest that patients with cerebellar lesions, acute or chronic, may be able to learn to improve their postural stability.

Adult↗
PubMed1977

Contralateral temporal horn widening in unilateral supratentorial mass lesions: a diagnostic sign indicating tentorial herniation.

Tentorial herniation secondary to supratentorial mass lesions will cause aqueductal compression and raised intraventricular pressure. Under ordinary circumstances, a raised intraventricular pressure will result in the development of hydrocephalus, but, in the presence of a large supratentorial mass, it is impossible for a generalized hydrocephalus to develop; only those parts of the ventricular system that are somehow shielded from the pressure effect of the mass lesion will be able to dilate. This dilatation will most frequently involve the contralateral temporal horn. Often, the atrium of the ventricle and the occipital horn will also be involved and, occasionally, the entire ventricle on the side opposite the mass will be dilated. The association of a large supratentorial mass with considerable shift of midline structures and computed tomography findings of a dilated contralateral temporal horn should be considered indicative of tentorial herniation, unless there are signs of preexisting atrophy or other preexisting disease processes that could have caused widening of the temporal horn.

Brain Diseases↗
PubMed1984

Cystic mural nodule in cerebellar hemangioblastoma: CT demonstration.

An example of a form of cystic hemangioblastoma of the cerebellum in which the mural nodule itself showed a central cystic area is presented. Although the precise etiology of the cystic component within hemangioblastoma tumor nodules is not known, it probably results from tumor degeneration. Computed tomography is valuable in identifying this feature, which, if present, is very suggestive of the diagnosis of hemangioblastoma. Other diagnostically useful CT features of hemangioblastoma that were present in this case included location of the mural nodule adjacent to the nearest pial surface, isodensity of the nodule on the preinfusion scan, and very rapid circulation of contrast material through the wall of the nodule, demonstrated by dynamic CT technique.

Adult↗