Late Onset Aqueductal Stenosis-associated Hydrocephalus Mimicking Pure Akinesia with Gait Freezing

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Evaluation of Late Onset Congenital Aqueductal Stenosis Hydrocephalus

Aqueductal stenosis is responsible for 20% of cases of hydrocephalus. Its incidence ranges from 0.5 to 1.0 in 1,000 births, with a recurrence risk in siblings of 1.0% to 4.5%. The onset of symptoms is usually insidious and can occur at any time from birth to adulthood. Associated malformations in neighboring structures are common The presence of these malformations helps to explain the intellec...

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Evaluation of Late Onset Congenital Aqueductal Stenosis Hydrocephalus

Aqueductal stenosis is responsible for 20% of cases of hydrocephalus. Its incidence ranges from 0.5 to 1.0 in 1,000 births, with a recurrence risk in siblings of 1.0% to 4.5%. The onset of symptoms is usually insidious and can occur at any time from birth to adulthood. Associated malformations in neighboring structures are common The presence of these malformations helps to explain the intellec...

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Pure akinesia with gait freezing: a clinicopathologic study

BACKGROUND Pure akinesia with gait freezing is a rare syndrome with few autopsied cases. Severe freezing of gait occurs in the absence of bradykinesia and rigidity. Most autopsies have revealed progressive supranuclear palsy. We report the clinical and postmortem findings of two patients with pure akinesia with gait freezing, provide video recordings of these patients, and review the literature...

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Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy.

The clinical syndrome of pure akinesia has most often been associated with progressive supranuclear palsy (PSP) and is characterized by difficulty initiating gait and "freezing" during walking, writing and speaking. Similar syndromes have been described under the rubrics of primary progressive freezing gait and primary gait ignition failure. We investigated the specificity of the clinical syndr...

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Clinical and single-photon emission computed tomography study of pure akinesia with freezing of gait.

The clinical syndrome of pure akinesia (PA) is considered the third phenotype of progressive supranuclear palsy (PSP), and is characterized by freezing of gait and prominent speech disturbance without rigidity or tremor. It is frequently considered one of the dopamine resistant motor syndromes, and its pathophysiology remains unclear. We report a patient followed in the Department of Neurology ...

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ژورنال

عنوان ژورنال: Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques

سال: 2016

ISSN: 0317-1671,2057-0155

DOI: 10.1017/cjn.2016.328