PISA Syndrome-Orthopedic manifestation of a neurological disease?
Published on: 26th May, 2020
OCLC Number/Unique Identifier: 8603897033
Pleurothotonus, commonly known as Pisa Syndrome (PS), is a rare neurological disorder characterized by lateral bending of the trunk with a tendency to lean to one side. This is typically mobile and resolves in supine position. It often presents as an incapacitating symptom of underlying neurodegenerative conditions like Parkinson’s disease, alzheimer’s disease, multisystem atrophy, dementia with Lewy bodies, progressive supranuclear palsy and even subacute sclerosing panencephalitis. It is known to be associated with neuroleptics, dopaminergic agents, valproic acid and lithium. PS is also seen in neurosurgical disorders like subdural hematoma, normotensive hydrocephalus, or as a late complication of pallidotomy in patients with PD. It can present either as an acute emergency or can develop gradually over time.PS tend to happen in coronal plane and can be controlled and managed if diagnosed in early stage. However, a chronic form known as “camptocormia” occurs often in a combined fashion with anteroposterior flexion which can improve to some extent, remain stable or even get worse. Pathophysiologic mechanism is not completely understood. This review will discuss all the updated literatures published in PS in terms of prevalence, pathophysiology, clinical manifestation, and treatment modalities.
Atypic subacute sclerosing panensefalitis in a six-year-old male
Published on: 23rd November, 2022
Subacute sclerosing panencephalitis is a rare, slow, and insidious neurodegenerative disease caused by measles. This disease mostly has a classic course. However, sometimes it can be presented with atypical manifestations. In this paper, we aim to present a six years old male patient that was hospitalized due to seizures and ataxia. Cerebral magnetic resonance imaging was normal on the first day of admission, but within a few days, the patient started to be apathetic. On the seventh day, magnetic resonance imaging showed hyperintense lesions in the thalamic, brainstem, and periventricular areas. Periodic epileptiform discharges were detected in the repeated electroencephalogram. Investigations from the cerebrospinal fluid showed markedly elevated measles virus IgG at 230U/ml consistent with the diagnosis of SSPE which should always be ruled out when a patient comes in with uncontrollable seizures, ataxia and apathy.
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