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The Cerebellar Ataxias and Hereditary Spastic Paraplegias
Published in John W. Scadding, Nicholas A. Losseff, Clinical Neurology, 2011
It is essential in late onset ataxia, where there is no family history, to exclude a posterior fossa structural lesion (such as a tumour or Chiari malformation), hydrocephalus, vitamin B12 deficiency (and vitamin E where there is any doubt), thyroid function tests and, where indicated (see below), a systemic malignancy. In most patients, these tests are all normal and a sporadic cerebellar degeneration is left as a diagnosis of exclusion. Within this group, five phenotypes are recognized: Dejerine–Thomas type: The onset is typically between 35 and 55 years of age. There is cerebellar ataxia, and varying combinations of dementia, parkinsonism, supranuclear eye movement abnormalities and areflexia. Optic atrophy or retinopathy are rare (Figure 21.3). Multiple system atrophy (MSA) may take the form of a late onset degenerative cerebellar ataxia as well as atypical parkinsonism or primary autonomic failure. A cerebellar syndrome is associated with parkinsonism, pyramidal signs and sometimes autonomic features such as postural hypotension, bladder dysfunction and impotence. The parkinsonism rarely responds well to levodopa. Nystagmus may occur but not severe gaze palsies, optic atrophy or retinopathy; dementia is not a feature of MSA. There is cerebellar and brainstem atrophy on MRI, which may also show an abnormal signal in the putamen. Denervation of the external urethral sphincter detected by electromyography is characteristic. Pathologically, there is degeneration of the cerebellum, brainstem (including substantia nigra), basal ganglia and sometimes the intermediolateral columns of the spinal cord with characteristic glial and neuronal cytoplasmic inclusions in these areas. Severe and early autonomic failure or bladder dysfunction is suggestive of MSA. Perhaps a quarter of those with the Dejerine–Thomas type of sporadic ataxia actually have MSA.Marie-Foix–Alajouanine type. These patients develop ataxia later, often after 55 years of age. There is unsteadiness of gait but less limb ataxia, corresponding to a degeneration of the cerebellar vermis.Dyssynergia cerebellaris progressiva. The onset of ataxia is between 40 and 60 years of age and is followed by increasingly severe tremor of the limbs (intention tremor with resting and postural elements).A rare type with bilateral vestibulopathy and peripheral neuropathy.
Prevalence of bilateral vestibulopathy among older adults above 65 years on the indication of vestibular impairment and the association with Dynamic Gait Index and Dizziness Handicap Inventory
Published in Disability and Rehabilitation, 2023
Katrine Storm Piper, Carsten Bogh Juhl, Hanne Elkjaer Andersen, Jan Christensen, Kasper Søndergaard
Bilateral vestibulopathy (BV) is a syndrome often leading to physical, cognitive, and emotional disabilities [1–3]. Causes and clinical signs of BV differ, especially among the older adults, and diagnosis is a challenge [1,4]. The frequency of BV increases with age, and the ageing population leads to an increasing number of older patients with BV [1,2]. The prevalence of BV among adult Americans is estimated to 28 out of 100,000 based on anamnestic diagnostics of people with symptoms of dizziness [2]. In Germany, the prevalence of BV is estimated to 3.6% (mean age 60.8 years) increasing to 16.1% among older adults above 79 years [1]. The cause of BV varies with more than 20 different etiologies including ototoxicity, genetic disorders, Ménière’s disease, infectious diseases, and neurodegenerative diseases and in 31–51% of BV patients the etiology of BV remains idiopathic [5].
Evaluation of high frequency horizontal VOR parameters in patients with chronic bilateral and unilateral peripheral vestibulopathy: a preliminary study
Published in Acta Oto-Laryngologica, 2020
Gulce Kirazli, Sevinc Hepkarsi, Tayfun Kirazli
The study has been conducted as prospective and taken place in ENT department. The study has been done by following the ethical principles of Helsinki Declaration. Both verbal and written informed consent have been obtained from all participants. Furthermore, the inclusion criteria of the study have not included history of acute, prolonged spontaneous vertigo; but it has consisted of a history of chronic imbalance, unilateral or bilateral total canal paresis (100%) on caloric testing and being 18–65 years old. Some participants with otological problems such as chronic otitis media, tympanic membrane perforation, outer ear atresia, metabolic disease, neurological, psychological and mental illness, severe visual impairment, and temporomandibular joint dysfunctions have been excluded from the study. The study has just included six patients with bilateral vestibulopathy, ten patients with unilateral vestibulopathy, and fifteen healthy asymptomatic controls.
Clinical application of MIIRMR as a salvage method in gadolinium-enhanced MRI after intra-tympanic injection
Published in Acta Oto-Laryngologica, 2022
Jianjian Huang, Cheng Tang, Wei Xia, Yiwei Feng, Songhua Tan, Lihong Xie, Muliang Jiang, Yuhong Qin, Huiting Zhang, Anzhou Tang
Using 3 D-FLAIR + 3 D-real IR + MIIRMR, EH was observed in 33/34 (97.06%) ipsilateral ears with definite MD, 9/28 (32.14%) contralateral ears with unilateral definite MD, and 2/4 (50.0%) ears with probable MD. In 38 ears with definite or probable MD, 3 D-FLAIR, 3 D-real IR, 3 D-FLAIR + 3 D-real IR, 3 D-FLAIR + MIIRMR, and 3 D-FLAIR + 3 D-real IR + MIIRMR detected 28, 28, 31,32 and 35 ears with EH, respectively, representing positive rates of 73.68%, 73.68%, 81.58%, 84.21%, and 92.11%, respectively. Among patients without MD, EH was detected in 1 ear with vestibular schwannoma, 1 ear of a patient with bilateral vestibulopathy, 1 ear of a patient with vestibular migraine, and 1 ear of an ipsilateral type of delayed endolymphatic hydrops.