Frontotemporal dementia as a comorbidity to idiopathic normal pressure hydrocephalus (iNPH): a short review of literature and an unusual case

Abstract

Behavioural variant frontotemporal dementia (bvFTD) and idiopathic normal pressure hydrocephalus (iNPH) are neurodegenerative diseases that can present with similar symptoms. These include decline in executive functions, psychomotor slowness, and behavioural and personality changes. Ventricular enlargement is a key radiological finding in iNPH that may also be present in bvFTD caused by the C9ORF72 expansion mutation. Due to this, bvFTD has been hypothesized as a potential comorbidity to iNPH but bvFTD patients have never been identified in studies focusing in clinical comorbidities with iNPH. Here we describe a patient with the C9ORF72 expansion-associated bvFTD who also showed enlarged ventricles on brain imaging. The main clinical symptoms were severe gait disturbances and psychiatric problems with mild cognitive decline. Cerebrospinal fluid removal increased the patient’s walking speed, so a ventriculoperitoneal shunt was placed. After insertion of the shunt, there was a significant improvement in walking speed as well as mild improvement in cognitive function but not in neuropsychiatric symptoms relating to bvFTD. Comorbid iNPH should be considered in bvFTD patients who have enlarged ventricles and severely impaired gait.

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment

“…The extrapyramidal symptoms of these diseases can mimic the symptoms of patients with NPH [36]. Even these patients can improve temporarily after VP-shunting [10]. It cannot be excluded, that some of our shunted patients were in the preclinical phase of the neurodegenerative disease at the time of surgery.…”

“…Common comorbidities among iNPH patients, arterial hypertension, diabetes mellitus, and vascular dementia, may be major prognostic factors [9]. Neurodegenerative diseases (dementia with Lewy bodies, Parkinson`s disease, Alzheimer's disease, progressive supranuclear palsy, frontotemporal dementia) could also present with extrapyramidal symptoms mimicking some symptoms of NPH [10,11]. Therefore, a comprehensive differential testing is required.…”

Predicting Development of Alzheimer’s Disease in Patients with Shunted Idiopathic Normal Pressure Hydrocephalus

“…A pathophysiological link has been proposed [43] and changes in amyloid β processing is detected [44] but CSF shunt does not improve symptoms of AD (without concomitant iNPH) [45]. For future prospects, ratio of Aβ42/40 should be evaluated with iNPH cohort [46] and prognosticators for other concomitant dementias should be examined [47].…”

Perceptions of mothers about the quality of life of children with hydrocephalus

“…A research performed with children with hydrocephalus corroborates these findings, since it has shown that motor skills can suffer losses at different levels, since of the five children evaluated, two demonstrated a greater impairment of motor function, while the others presented greater neuromotor ability (16) . It should be emphasized that there may be improvement of this motor function from the treatment and care performed (17) .…”