Psychotic behavior in normal pressure hydrocephalus

Abstract

Normal pressure hydrocephalus (NPH) usually presents with progressive gait impairment, urinary incontinence, and cognitive deficits. Many studies have shown that NPH can mimic other disorders like Parkinson disease with tremors and rigidity. In this report, we present our experience with three patients who had psychotic behavior and were treated with psychiatric medications but were subsequently investigated and found to have NPH. After lumboperitoneal shunting, all the three became normal and were even able to stop the psychiatric medications completely. The message is that NPH can mimic a psychiatric disorder and awareness about this well treatable condition is important especially among the psychiatrists and geriatricians.

INTRODUCTION

Normal pressure hydrocephalus (NPH) is commonly being diagnosed because of the increasing aging population and is an uncommon cause of a reversible neurological condition. The gait disturbance, cognitive and urinary symptoms, and dilated ventricles on imaging are all well established.[1] However, NPH could mimic many other neurological disorders including Parkinson’s disease,[2] about which we had presented earlier. Similarly, aggressive behavior, apathy, and other psychotic symptoms have been shown.[1] Even though cognitive and mental symptoms are a prominent part of the clinical picture of NPH, delusions can usually be a prominent early feature, combined with slowing of mental and physical activity, which may lead to a diagnosis of delusional disorder, schizophrenia, depression, or dementia.[3]

CASE SERIES

Patient 1 is a 78-year-old male and was observed by the family approximately 3 years ago to have become inactive, apathetic with visual hallucinations, and persecutory delusions. He would arrange and rearrange the furniture in the living room and would be muttering to self. He was put on medications, but his condition continued to progress, and he became confused, apathetic, and mute and was unable to walk. Clinical assessment revealed UMN signs with hyperreflexia and extensor plantar. MRI brain revealed dilated lateral and third ventricles with crowding of Sulci. A diagnosis of normal pressure hydrocephalus was made, and the patient underwent a trial lumbar puncture drainage, which was highly positive. He underwent a lumboperitoneal shunt following which symptoms disappeared, leading to complete recovery. He is now completely off psychiatric medicines.

Patient 2, a 69-year-old lady presented with hallucinations with irrelevant talk and also excessive anger and aggressive behavior. Progressively, she started having difficulty in walking and also became incontinent. Clinical examination in our center showed magnetic gait with cognitive decline. A diagnosis of normal pressure hydrocephalus was made, and the patient underwent a trial lumbar puncture drainage which was highly positive. She underwent a lumboperitoneal shunt, after which her hallucinations and talk became normal with reduced anger episodes. The psychiatric team is gradually tapering off all psychiatric medications. Her gait and cognitive function has almost become normal, and she is fully continent.

Patient 3 is a 65-year-old lady who had hallucinations and anger episodes with refusal to eat. She was also confirmed to be having normal pressure hydrocephalus. She also had the LP trial and then lumboperitoneal shunt. Now she is totally off all psychiatric medications, and her motor functions have improved by 70 to 80%. The family claims that they notice progressive improvement every passing day.

DISCUSSION

NPH is a common cause of potentially reversible dementia. It can present with psychiatric manifestations that may hinder its diagnosis. Pujol et al.[4] presented five case studies having NPH with psychiatric presentation and concluded that NPH can present with predominant psychiatric symptomatology, which can precede the appearance of other symptoms. Similarly, Rice and Gendelman[5] published five cases with NPH with psychiatric manifestations. A study conducted by Oliveira et al.[6] showed that psychiatric symptoms could occur in the early course of NPH and can be seen in up to 71% of these patients, especially anxiety, depression, and psychotic syndromes; personality changes; anxiety; depression; psychotic syndromes; obsessive compulsive disorder; Othello syndrome; shoplifting; and mania.

In this series, detailed neurological examination followed by MRI and then trial LP CSF drainage by experienced neurosurgeons was done in all cases and all patients had good improvement in their symptoms (both motor and psychiatric) following the trial. Interestingly, all three soon came back to the pre-LP status within a few days, thus providing a double confirmation.

Most observations of psychiatric sequelae of NPH have been limited to case reports and have included frontal lobe symptoms such as apathy and akinetic mutism as well as reports of mania, aggression, mood cycling, and psychosis.[3,4,5] Sometimes, even response to typical treatment in psychiatric syndromes may be impaired, with intolerance to even low doses of antipsychotics, who were subsequently revealed to be having communicating normal pressure hydrocephalus.[3] Cromwell, Tew, and Mark[7] reported two cases with aggressive behavior which improved after a shunt surgery was performed. In our cases also, the patient with aggressive behavior calmed down over a few weeks so that all psychiatric medications could be stopped.

Late onset psychosis was the primary diagnosis in these patients. But in two of them, there was no change inspite of prolonged antipsychotics, and in, the elderly male actually, symptoms worsened with introduction of antipsychotics. So, the diagnosis and treatment were reviewed and surgery was done. This is a case note retrospective study. In this case, all the three patients improved from their neurological and psychiatric symptoms during follow-up. This fact helped us to rule out other conditions like organic psychosis, Parkinson Plus syndromes, or Lewy body dementia. Lewy body dementia is a postmortem diagnosis.

Alegeti S and Burson[8] had reviewed the available case reports until May 2015 and could identify only 11 cases in the literature prior to their case. One of these patients had good improvement in psychosis post surgery and could discontinue neuroleptics.

The traditional treatment for NPH has been ventriculoperitoneal shunting.[9] More than 60% of patients show very good improvement after shunting. But ventriculoperitoneal shunt (VP) has the risk of causing brain injury. So lumboperitoneal (LP) shunt is a very good and equally effective option in such cases.[10] A similar view was also expressed by Kanemoto et al.[11] They analyzed 22 cases who underwent LP shunt and found that the effect of shunt on neuropsychiatric symptoms was confirmed in 22 NPH patients. Apathy and depression significantly improved after shunt surgery, and this helped in reducing the caregiver burden.

Improvement of psychosis with CSF shunting procedures supports causal relation between NPH and psychosis. All our patients qualified for diagnosis of NPH as per the consensus criteria. They also exhibited poor tolerance and even worsening of symptoms on the addition of antipsychotics. However, with the surgical intervention, not only did all three patients completely recover but also their antipsychotics are being tapered and stopped. These cases illustrate the importance of considering a diagnosis of NPH in any elderly patient who presents with psychiatric symptoms at the earliest and going forward to investigate both clinically and radiologically.

NPH mimics many other neurodegenerative conditions like Parkinson’s disease (2) and even psychiatric problems (4,5,6,8) and is a well-treatable condition. We thought it our duty to bring again this issue to the attention of the medical fraternity so that patients are given the correct diagnosis and treatment. In this series, all the elderly patients had upper motor signs like brisk reflexes and extensor plantar. The take home message is that, especially in elderly, it would be a good idea to just check the deep tendon reflexes and the plantar reflex, especially in elderly late onset psychosis. This may be followed up with MRI. Of course, It would be ideal to do MRI in all cases.

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Conflicts of interest

There are no conflicts of interest.