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Pathology Report on Intracranial Shunt for Treatment of NPH

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Pathology Report on Intracranial Shunt for Treatment of NPH

Normal pressure hydrocephalus (NPH) is a serious and complex neurological disease that

presents and culminates in therapeutic and diagnostic challenges for physicians. For instance,

NPH is associated with challenges in differential diagnosis with neurodegenerative disorders,

culminating in lack of treatment or its delay hindering patient recovery often with dramatic

consequences in terms of surging socio-economic costs and clinical deterioration. Besides,

NPH’s pathophysiology and diagnosis contribute to its emerging challenges (Oliveira et al.,

2022). The disorder presents inconsistency in its clinical progression and presentation. It is akin

to or occur in conjunction with disorders prevalent in older populations, including Parkinson’s,

Alzheimer’s, senile dementia, lumbar stenosis, urological, and cerebrovascular diseases. In

addition, its pathophysiology remains debatable, with some researchers claiming it stems from

periventricular white matter and the degeneration of its vascular supply (Trungu et al., 2022).

Although radiological and clinical examinations are the primary diagnostic methods for NPH,

they exhibit high specificity and sensitivity variability often linked to heterogeneity of the

examination methods used. This pathology report examines NPH particularly focusing on the

employment of intracranial shunts, including ventriculoperitoneal shunts (VPS), as the primary

its primary treatment option. The aim is to address NPH’s epidemiology, diagnostic criteria,

etiology, pathology, and neuromedicine’s role in its management, including common treatment

modalities and their associated complications.

Pathology Definition

Normal-pressure hydrocephalus (NPH) is a serious and complex neurological disease that

presents and culminates in therapeutic and diagnostic challenges for physicians. According to

Patel et al. (2023), NPH is neurological disease that becomes apparent via the abnormal
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cerebrospinal fluid (CSF) accumulation within brain ventricles, culminating in ventriculomegaly

– the enlargement of brain ventricles because of CSF buildup. This occurs because CSF

accumulation leads to a surge in intracranial pressure that compresses an individual’s adjacent

brain tissue. Typically, this neurological disorder manifests in a triad of clinical symptoms,

encompassing urinary incontinence, gait disturbances, and cognitive impairment (Patel et al.,

2023).

Two primary categories of NPH abound. They include idiopathic NPH (iNPH) and

secondary NPH (sNPH) (Oliveira, 2019). The former class demarcates all cases where the

underlying ventriculomegaly remains a mystery and is prevalent in older adults. Besides, iNPH

is associated with urinary dysfunction, gait impairment, and progressive cognitive decline. On

the other hand, secondary normal-pressure hydrocephalus (sNPH) includes all NPH cases

whereby the inciting event or identifiable underlying cause precedes NPH development. Some of

the potential causes include intracranial malignancies, head trauma, subarachnoid hemorrhage,

and various factors that affect cerebrospinal fluid dynamics.

Symptoms of NPH

A triad of symptoms characterized NPH. They include impaired bladder control,

cognitive impairment, and gait disturbance (difficulty walking) (“What is normal pressure

hydrocephalus?” 2023). Although not simultaneous in their occurrence, the symptoms can

manifest at different NPH stages with varying severity levels. Regarding gait disturbances or

difficulty walking, they range in terms of their severity from mere mild imbalance to the inability

to walk or stand. The gait disturbance presents as shuffling, short-stepped (wide-based), and

typically slow in character. Individuals can be predisposed to falls with surging frequency, with

individuals developing problems descending surfaces or stairs. Individuals often make turns with
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hesitancy and a series of choppy steps in their path, with a tendency for unintentional

acceleration and difficulty maintaining a uniform walking pace. In addition, individuals suffer

“magnetic” gait (“freezing of gait”) where they experience heavy feet and find it problematic to

move or initiate normal strides (“What is normal pressure hydrocephalus?” 2023). It is

noteworthy that gait disturbance is often the first symptom to become apparent and can be the

most troubling. On the other hand, cognitive impairments (mild dementia) manifests as short-

term memory loss, problems multi-tasking or making decisions, difficulty handling routine tasks,

mood changes, forgetfulness, and loss of interest in routine or daily activities. Lastly, impairment

in bladder control (urinary incontinence features urinary urgency and frequency in mild cases

while in severe cases the individual suffers urinary incontinence or completely loses bladder

control. However, some individuals with NPH do not exhibit signs or symptoms of bladder

issues.

Etiology of NPH

NPH’s etiology is typically multifactorial, with various factors involved in its

development and progression. Some of the prominent factors involved included impaired

absorption of cerebrospinal fluid, altered cerebral perfusion, altered cerebrospinal fluid

dynamics, and associated glymphatic system (Patel et al., 2023). One of the primary factors in

NPH development and progression is the impaired absorption of CSF via the arachnoid villi in

dural sinuses. Venous hypertension linked to various causes, including intracranial malignancies

and subarachnoid hemorrhage can prevent CSF from being normally absorbed and culminate in

its accumulation. Altered cerebral perfusion is another vital factor in the pathogenesis of NPH.

The ventriculomegaly experienced in NPH can culminate in surging intracranial pressure,

causing global cerebral blood flow to decrease (Patel et al., 2023). Regional drops in cerebral
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blood flow often manifest in certain areas of the brains such as thalami, basal ganglia,

periventricular white matter, and frontal cortex. Concerning altered CSF dynamics, NPH is

linked to CSF dynamics alterations, resulting in CSF buildup within the ventricles. The

occurrence can emerge from a decrease in CSF production, a surge in CSF production or both.

Hence, altered dynamics can aggravate ventriculomegaly while affecting the normal CSF

exchange. Lastly, associated glymphatic system is thought to cause NPH development. Recent

studies suggest a link between NPH and this system, which is the central nervous system’s waste

clearance pathway. Disruptions in the flow of the associated glymphatic system may be

contributing factors to protein and waste product accumulation within the brain, potentially

resulting in cognitive impairment and other NPH symptoms.

Common Detection, Diagnosis, and Staging

Physicians use a range of clinical evaluation methods, diagnostic tests, and tools to

diagnose and stage NPH. Diagnosing NPH clinically entails conducting a thorough evaluation of

the patient’s neurological examination, medical history, and reviewing their presenting

symptoms (Oliveira et al., 2019). The hallmark of NPH is the classic triad of urinary

incontinence, gait disturbance, and cognitive impairment. Besides clinical evaluation, physicians

also perform neuroimaging procedures, especially magnetic resonance imaging (MRI), which

has a high efficacy in detecting and diagnosing NPH. Clinics adhere to specific MRI criteria for

diagnosing NPH, including flow void in the cerebral aqueduct, changes in periventricular white

matter, and ventriculomegaly proportion to cerebral atrophy. Other neuroimaging characteristics

may encompass disproportionately enlarged subarachnoid space hydrocephalus (DESH) and an

Evans Index that exceeds 0.3 (Oliveira et al., 2023). Moreover, hospitals also use a series of

diagnostic tests to diagnose NPH, including large-volume or tap test lumbar puncture. These
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tests aim to examine the response of NPH symptoms to temporary removal of CSF. The tap tests

can predict the plausibility of improvement with shunt surgery while helping differentiate NPH

from other conditions presenting similar symptoms (“What is NPH?” 2023). Staging of NPH, in

most cases, entails the assessment of symptom severity and pinpointing causes contributing to

the disorder. Thus, staging and evaluation are vital phases in determining the appropriate course

of treatment and monitoring disease progression in patients.

Epidemiology, Morbidity, and Mortality Rates of NPH

Idiopathic normal pressure hydrocephalus (iNPH) represents a complex neurological

condition characterized by an abnormal accumulation of cerebrospinal fluid within the brain's

ventricles, resulting in a triad of clinical symptoms: gait disturbance, cognitive impairment, and

urinary incontinence. Epidemiological data underscores the significance of this condition,

particularly among the elderly population. Studies suggest that the prevalence of iNPH ranges

from 1.4% to 3.7% in a population-based setting among individuals over the age of 65, with a

notable increase to 5.9% to 8.9% in those over 80 years old (Fang et al., 2022). Moreover, the

prevalence of iNPH appears to have experienced an upward trajectory over the years, with an

increase from 0.04% in 2007 to 0.2% in 2017 in the United States among inpatients aged 60 and

above, indicative of a growing health concern that demands attention.

The impact of iNPH on individuals' health is substantial, as it can significantly affect

their quality of life and lead to notable morbidity. While direct mortality rates attributed to iNPH

remain relatively low, the long-term consequences of the condition can result in significant

disability and reduced cognitive function. This underscores the critical importance of early

diagnosis and appropriate management to mitigate the potentially debilitating effects of iNPH.
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Implementing timely interventions such as ventriculoperitoneal shunts has shown promise in

improving outcomes and reducing the associated morbidity.

Given the rising prevalence of iNPH in conjunction with the increasing life expectancy of

the global population, a comprehensive understanding of the epidemiology, mortality, and

morbidity of iNPH is crucial. Such insights serve as a basis for the development of more targeted

diagnostic and therapeutic strategies to effectively manage and improve the overall outcomes of

individuals affected by this condition. Continuous research efforts aimed at enhancing early

detection methods and optimizing treatment modalities are pivotal in alleviating the burden

posed by iNPH on both patients and healthcare systems worldwide. By addressing the

multifaceted challenges associated with iNPH, healthcare professionals and policymakers can

foster improved care and better quality of life for individuals affected by this condition.

Neuromedicine Involvement in NPH Management

Effectively managing NPH requires physicians to employ a multidisciplinary approach

that encompasses various NPH specialists, such as neurosurgeons, neuroradiologists, physical

therapists, neuropsychologists, and neurologists. This team of specialists is vital in ensuring that

patients are accurately diagnosed, comprehensively assessed, and treated for their condition.

Neuromedicine specialists play a significant role in managing NPH, especially in the monitoring

of disease progression, analyzing gait, and evaluating cognitive function. They also evaluate the

effectiveness and efficacy of treatment modalities, such as the employment of intracranial shunts

while providing valuable insights into the long-term NPH management.

Common Treatment Modalities

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Various treatment modalities exist for NPH. They include ventriculoperitoneal shunting,

endoscopic ventriculostomy, and acetazolamide administration. Ventriculoperitoneal shunting is

the primary NPH treatment modality. It aims to divert excess cerebrospinal fluid from brain

ventricles to the peritoneal cavity, helping reduce intracranial pressure in the brain while

alleviating associated symptoms, such as gait disturbances and cognitive impairment (Trungu et

al., 2022). Although this shunting treatment modality has high efficacy, it has prospects of

complications, which physicians must carefully consider during patient counselling and

treatment planning (Fereshtehnejad et al., 2023). Researchers and clinicians have also explored

alternative NPH treatment methods, such as acetazolamide administration and endoscopic third

ventriculostomy. Despite these alternative modalities offering reprieve for designated patient

populations, evidence corroborating their widespread use remain scanty, accentuating the need

for further clinical trials and research to determine their safety and efficacy.

Conclusion

Overall, NPH remains among the complex neurological conditions that necessitate a

thorough understanding of their underlying pathology and appropriate management strategies.

The employment of intracranial shunts – ventriculoperitoneal shunts – has emerged as the

primary NPH treatment modality with high efficacy for alleviating all NPH -related symptoms.

Although the diagnostic challenges and potential challenges linked to NPH treatment persist,

using a multidisciplinary treatment approach encompassing a team of neuromedicine specialists

is vital for improving individuals’ overall quality of life and optimizing patient outcomes.

Further clinical trials and research are warranted to determine more definitive guidelines for use

of alternative NPH modalities and intracranial shunts. Besides, it is critical to ensure patients
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have a comprehensive understanding of potential treatment complications to ensure long-term

outcomes and informed patient care.

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References

Fang, X., Deng, Y., Xu, X., Shu, W., Tang, F., Li, S., Zhu, T., Zhang, L., Zhong, P., & Mao, R.

(2022). One-year outcome of a lumboperitoneal shunt in older adults with idiopathic

normal pressure hydrocephalus. Frontiers in

Surgery, 9. https://doi.org/10.3389/fsurg.2022.977123

Fereshtehnejad, S., Nicholls, M., Huang, S., & Akter, R. (2023). Normal pressure

hydrocephalus treated with ventriculoperitoneal shunt. Canadian Medical Association

Journal, 195(3), E115-E119. https://doi.org/10.1503/cmaj.221027

Oliveira, L. M., Nitrini, R., & Román, G. C. (2019). Normal-pressure hydrocephalus: A critical

review. Dementia & Neuropsychologia, 13(2), 133-143. https://doi.org/10.1590/1980-

57642018dn13-020001

Patel, S., Ditamo, M., Mangal, R., Gould, M., & Ganti, L. (2023). Normal pressure

hydrocephalus. Cureus. https://doi.org/10.7759/cureus.35131

Trungu, S., Scollato, A., Ricciardi, L., Forcato, S., Polli, F. M., Miscusi, M., & Raco, A. (2022).

Clinical outcomes of shunting in normal pressure hydrocephalus: A multicenter

prospective observational study. Journal of Clinical Medicine, 11(5),

1286. https://doi.org/10.3390/jcm11051286

What is normal pressure hydrocephalus (NPH)? (2023, October 10). Hydrocephalus

Association. https://www.hydroassoc.org/about-normal-pressure-hydrocephalus/