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Normal Pressure Hydrocephalus

Normal pressure hydrocephalus Hydrocephalus Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial. Subarachnoid Hemorrhage (NPH) is a neurodegenerative disorder featuring enlarged ventricles with normal opening pressures on lumbar puncture Lumbar Puncture Febrile Infant. Clinically, the condition is characterized by the triad of gait Gait Manner or style of walking. Neurological Examination abnormalities, dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person's memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders, and urinary urgency or incontinence. Normal pressure hydrocephalus Hydrocephalus Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial. Subarachnoid Hemorrhage can be either idiopathic Idiopathic Dermatomyositis or secondary to intraventricular or subarachnoid hemorrhage Subarachnoid Hemorrhage Subarachnoid hemorrhage (SAH) is a type of cerebrovascular accident (stroke) resulting from intracranial hemorrhage into the subarachnoid space between the arachnoid and the pia mater layers of the meninges surrounding the brain. Most SAHs originate from a saccular aneurysm in the circle of Willis but may also occur as a result of trauma, uncontrolled hypertension, vasculitis, anticoagulant use, or stimulant use. Subarachnoid Hemorrhage. Symptoms may be similar to those of Alzheimer and Parkinson's diseases. Diagnosis of NPH is clinical, in addition to lumbar puncture Lumbar Puncture Febrile Infant testing and neuroimaging Neuroimaging Non-invasive methods of visualizing the central nervous system, especially the brain, by various imaging modalities. Febrile Infant. Management is with surgical shunt placement to drain excess CSF from the cerebral ventricles.

Last updated: Jun 23, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Overview

Definition

Normal pressure hydrocephalus Hydrocephalus Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial. Subarachnoid Hemorrhage (NPH) is a neurodegenerative disorder characterized by the triad of dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders, progressive gait Gait Manner or style of walking. Neurological Examination abnormalities, and urinary urgency or incontinence.

Epidemiology

  • Most common form of hydrocephalus Hydrocephalus Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial. Subarachnoid Hemorrhage in adults
  • Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency: 2–20 cases per million individuals per year
  • Prevalence Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. Measures of Disease Frequency:
    • Secondary NPH: Prevalence Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. Measures of Disease Frequency is not determined by age.
    • Idiopathic Idiopathic Dermatomyositis NPH: Prevalence Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. Measures of Disease Frequency increases with age (usually > 60 years).
      • 0.2% in individuals aged 70–79
      • 6% in individuals > age 80
  • Represents approximately 6% of all cases of dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders
  • No sex Sex The totality of characteristics of reproductive structure, functions, phenotype, and genotype, differentiating the male from the female organism. Gender Dysphoria predilection

Etiology

  • Idiopathic Idiopathic Dermatomyositis: no identifiable causes
  • Secondary:
    • Intraventricular and subarachnoid hemorrhage Subarachnoid Hemorrhage Subarachnoid hemorrhage (SAH) is a type of cerebrovascular accident (stroke) resulting from intracranial hemorrhage into the subarachnoid space between the arachnoid and the pia mater layers of the meninges surrounding the brain. Most SAHs originate from a saccular aneurysm in the circle of Willis but may also occur as a result of trauma, uncontrolled hypertension, vasculitis, anticoagulant use, or stimulant use. Subarachnoid Hemorrhage (most common)
    • Acute or chronic meningitis Meningitis Meningitis is inflammation of the meninges, the protective membranes of the brain, and spinal cord. The causes of meningitis are varied, with the most common being bacterial or viral infection. The classic presentation of meningitis is a triad of fever, altered mental status, and nuchal rigidity. Meningitis
    • Traumatic brain injury Traumatic brain injury A form of acquired brain injury which occurs when a sudden trauma causes damage to the brain. Le Fort Fractures (TBI)
    • Paget disease (rare)

Pathophysiology

The pathophysiology of NPH is still unclear.

  • Possible contributing mechanisms:
    • Subclinical congenital Congenital Chorioretinitis hydrocephalus Hydrocephalus Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial. Subarachnoid Hemorrhage
    • Hyperdynamic flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure of CSF
    • Reduced compliance Compliance Distensibility measure of a chamber such as the lungs (lung compliance) or bladder. Compliance is expressed as a change in volume per unit change in pressure. Veins: Histology of the subarachnoid space Subarachnoid space The space between the arachnoid membrane and pia mater, filled with cerebrospinal fluid. It contains large blood vessels that supply the brain and spinal cord. Subarachnoid Hemorrhage
    • Decreased reabsorption of CSF
    • Reduced cerebral blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure 
    • Increased central venous pressure Central venous pressure The blood pressure in the central large veins of the body. It is distinguished from peripheral venous pressure which occurs in an extremity. Central Venous Catheter
  • Consequences:
    • Accumulation of CSF in the cranial vault Cranial Vault Increased Intracranial Pressure (ICP)
    • Increased intracranial pressure Increased Intracranial Pressure Normal intracranial pressure (ICP) is defined as < 15 mm Hg, whereas pathologically increased ICP is any pressure ≥ 20 mm Hg. Increased ICP may result from several etiologies, including trauma, intracranial hemorrhage, mass lesions, cerebral edema, increased CSF production, and decreased CSF absorption. Increased Intracranial Pressure (ICP) ( ICP ICP Normal intracranial pressure (ICP) is defined as < 15 mm Hg, whereas pathologically increased ICP is any pressure ≥ 20 mm Hg. Increased ICP may result from several etiologies, including trauma, intracranial hemorrhage, mass lesions, cerebral edema, increased CSF production, and decreased CSF absorption. Increased Intracranial Pressure (ICP)) → compression Compression Blunt Chest Trauma of corticospinal tract fibers (close to the lateral ventricles Lateral ventricles Cavity in each of the cerebral hemispheres derived from the cavity of the embryonic neural tube. They are separated from each other by the septum pellucidum, and each communicates with the third ventricle by the foramen of monro, through which also the choroid plexuses (choroid plexus) of the lateral ventricles become continuous with that of the third ventricle. Ventricular System: Anatomy) and brain stem Brain Stem The brain stem is a stalk-like structure that connects the cerebrum with the spinal cord and consists of the midbrain, pons, and medulla oblongata. It also plays a critical role in the control of cardiovascular and respiratory function, consciousness, and the sleep-wake cycle. Brain Stem: Anatomy (pedunculopontine nucleus Nucleus Within a eukaryotic cell, a membrane-limited body which contains chromosomes and one or more nucleoli (cell nucleolus). The nuclear membrane consists of a double unit-type membrane which is perforated by a number of pores; the outermost membrane is continuous with the endoplasmic reticulum. A cell may contain more than one nucleus. The Cell: Organelles) → gait Gait Manner or style of walking. Neurological Examination abnormalities
    • Enlargement of ventricles → poor interstitial perfusion and ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage → pushing of cerebral cortex Cerebral cortex The cerebral cortex is the largest and most developed part of the human brain and CNS. Occupying the upper part of the cranial cavity, the cerebral cortex has 4 lobes and is divided into 2 hemispheres that are joined centrally by the corpus callosum. Cerebral Cortex: Anatomy against the bones of the calvarium Calvarium Skull: Anatomy dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders
    • Compression Compression Blunt Chest Trauma of periventricular sacral fibers of the corticospinal tract → loss of supraspinal control of bladder Bladder A musculomembranous sac along the urinary tract. Urine flows from the kidneys into the bladder via the ureters, and is held there until urination. Pyelonephritis and Perinephric Abscess contraction → urinary incontinence Urinary incontinence Urinary incontinence (UI) is involuntary loss of bladder control or unintentional voiding, which represents a hygienic or social problem to the patient. Urinary incontinence is a symptom, a sign, and a disorder. The 5 types of UI include stress, urge, mixed, overflow, and functional. Urinary Incontinence

Clinical Presentation and Diagnosis

Family members or caretakers may report personality and/or behavioral changes and frequent urination or incontinence.

Clinical presentation

  • Classic triad of symptoms:
    • Progressive gait Gait Manner or style of walking. Neurological Examination abnormalities
    • Dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders
    • Urinary urgency and/or incontinence
    • Mnemonics:
      • “Wet, wacky, and wobbly” = incontinence, dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders, and gait Gait Manner or style of walking. Neurological Examination dysfunction
      • “AID” = ataxia Ataxia Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. Ataxia-telangiectasia, incontinence, and dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders
  • Gait Gait Manner or style of walking. Neurological Examination abnormalities:
    • 1st and most common symptom
    • Shuffling or wide-based gait Gait Manner or style of walking. Neurological Examination
    • External rotation Rotation Motion of an object in which either one or more points on a line are fixed. It is also the motion of a particle about a fixed point. X-rays of feet
    • Multiple-step turns
    • Freezing of gait Gait Manner or style of walking. Neurological Examination 
  • Dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders:
    • Executive dysfunction
      • Complex processes that require integration of complex, goal-oriented tasks and behaviors
      • Poor judgment Judgment The process of discovering or asserting an objective or intrinsic relation between two objects or concepts; a faculty or power that enables a person to make judgments; the process of bringing to light and asserting the implicit meaning of a concept; a critical evaluation of a person or situation. Psychiatric Assessment
      • Disorganization
      • Socially inappropriate behavior
    • Psychomotor retardation
    • Reduced attention Attention Focusing on certain aspects of current experience to the exclusion of others. It is the act of heeding or taking notice or concentrating. Psychiatric Assessment and concentration
    • Apathy Apathy Lack of emotion or emotional expression; a disorder of motivation that persists over time. Wernicke Encephalopathy and Korsakoff Syndrome
  • Urinary urgency and/or incontinence:
    • Gait Gait Manner or style of walking. Neurological Examination abnormality may interfere with getting to the bathroom with urgency.
    • Later stages → lack concern about incontinence
  • Symptoms not found with NPH that are seen with increased ICP ICP Normal intracranial pressure (ICP) is defined as < 15 mm Hg, whereas pathologically increased ICP is any pressure ≥ 20 mm Hg. Increased ICP may result from several etiologies, including trauma, intracranial hemorrhage, mass lesions, cerebral edema, increased CSF production, and decreased CSF absorption. Increased Intracranial Pressure (ICP):
    • Headaches
    • Nausea Nausea An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. Antiemetics and vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia
    • Visual loss
    • Papilledema Papilledema Swelling of the optic disk, usually in association with increased intracranial pressure, characterized by hyperemia, blurring of the disk margins, microhemorrhages, blind spot enlargement, and engorgement of retinal veins. Chronic papilledema may cause optic atrophy and visual loss. Idiopathic Intracranial Hypertension

Diagnosis

  • History and physical exam:
  • Lab testing—rule out treatable causes of dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders:
    • Vitamin B12 level
    • Thyroid Thyroid The thyroid gland is one of the largest endocrine glands in the human body. The thyroid gland is a highly vascular, brownish-red gland located in the visceral compartment of the anterior region of the neck. Thyroid Gland: Anatomy function tests
  • Large volume lumbar puncture Lumbar Puncture Febrile Infant (LP; tap test):
    • Document gait Gait Manner or style of walking. Neurological Examination function before LP:
      • Gait Gait Manner or style of walking. Neurological Examination speed
      • Stride length
      • Number of steps it takes to turn 360 degrees
      • Videotape may be helpful to compare with function after LP.
    • Normal opening pressure (6–20 cm H2O) is seen with NPH.
    • Remove 30–50 mL of CSF.
    • Documented improvement in gait Gait Manner or style of walking. Neurological Examination function 30–60 minutes after procedure suggests improved outcome after ventriculoperitoneal (VP) shunt.
  • Imaging:
    • X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests: Consider cervical or lumbar spinal X-rays X-rays X-rays are high-energy particles of electromagnetic radiation used in the medical field for the generation of anatomical images. X-rays are projected through the body of a patient and onto a film, and this technique is called conventional or projectional radiography. X-rays to evaluate for spondylosis Spondylosis A degenerative spinal disease that can involve any part of the vertebra, the intervertebral disk, and the surrounding soft tissue. Central Cord Syndrome causing gait Gait Manner or style of walking. Neurological Examination dysfunction.
    • MRI of the brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification:
      • Ventriculomegaly Ventriculomegaly Hydrocephalus in Children
      • Characteristic high signal periventricular white matter White Matter The region of central nervous system that appears lighter in color than the other type, gray matter. It mainly consists of myelinated nerve fibers and contains few neuronal cell bodies or dendrites. Brown-Séquard Syndrome changes
      • Enlarged subarachnoid space Subarachnoid space The space between the arachnoid membrane and pia mater, filled with cerebrospinal fluid. It contains large blood vessels that supply the brain and spinal cord. Subarachnoid Hemorrhage
      • Sylvian fissure Fissure A crack or split that extends into the dermis Generalized and Localized Rashes widening
      • CSF flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure rate > 24.5 mL/min is 95% specific for NPH.
Illustration of dw-mri changes location

MRI of normal pressure hydrocephalus Hydrocephalus Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, intracranial. Subarachnoid Hemorrhage (NPH):
Note the widening of the ventricles, which is out of proportion to the degree of cerebral atrophy Cerebral Atrophy Subdural Hemorrhage.

Image: “Illustration of DW-MRI changes location” by . License: CC BY 4.0, cropped by Lecturio.

Management and Prognosis

Management

The treatment of NPH is with an implanted ventricular shunt.

  • Types of shunts:
    • Ventriculoperitoneal (VP): surgically created communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence between the lateral ventricle and the peritoneal cavity Peritoneal Cavity The space enclosed by the peritoneum. It is divided into two portions, the greater sac and the lesser sac or omental bursa, which lies behind the stomach. The two sacs are connected by the foramen of winslow, or epiploic foramen. Peritoneum: Anatomy
    • Ventriculoatrial: catheter from the lateral ventricle into the heart
  • Predictors of improvement with shunts:
    • Early appearance of gait Gait Manner or style of walking. Neurological Examination disorder
    • Gait Gait Manner or style of walking. Neurological Examination disorder most prominent symptom
    • Duration of symptoms < 6 months
    • Clinical response to CSF removal by LP
    • Absence of negative prognostic indicators:
      • Early appearance of dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders
      • Dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders for > 2 years
      • Alcoholism Alcoholism A primary, chronic disease with genetic, psychosocial, and environmental factors influencing its development and manifestations. The disease is often progressive and fatal. It is characterized by impaired control over drinking, preoccupation with the drug alcohol, use of alcohol despite adverse consequences, and distortions in thinking, most notably denial. Each of these symptoms may be continuous or periodic. Wernicke Encephalopathy and Korsakoff Syndrome
  • Complications of shunts:
    • Overdrainage:
      • Most common complication in 1st year
      • May be asymptomatic or may present with headaches
      • Radiologic signs: subdural effusions; subdural hematoma Hematoma A collection of blood outside the blood vessels. Hematoma can be localized in an organ, space, or tissue. Intussusception due to tearing of the bridging veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins: Histology
    • Infection
    • Seizures Seizures A seizure is abnormal electrical activity of the neurons in the cerebral cortex that can manifest in numerous ways depending on the region of the brain affected. Seizures consist of a sudden imbalance that occurs between the excitatory and inhibitory signals in cortical neurons, creating a net excitation. The 2 major classes of seizures are focal and generalized. Seizures
    • Intracerebral hemorrhage Intracerebral Hemorrhage Intracerebral hemorrhage (ICH) refers to a spontaneous or traumatic bleed into the brain parenchyma and is the 2nd-most common cause of cerebrovascular accidents (CVAs), commonly known as stroke, after ischemic CVAs. Intracerebral Hemorrhage during catheter placement in 3%–6% of cases
    • Blocked shunt
    • Arrhythmias during catheter placement in ventriculoatrial shunts

Prognosis Prognosis A prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas

  • Individuals have an 80%–90% chance of improvement in all symptoms after surgery.
  • Worse outcomes are seen with:
    • Severe dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders or dementia Dementia Major neurocognitive disorders (NCD), also known as dementia, are a group of diseases characterized by decline in a person’s memory and executive function. These disorders are progressive and persistent diseases that are the leading cause of disability among elderly people worldwide. Major Neurocognitive Disorders as the presenting symptom
    • Concomitant cerebrovascular disease
    • Cerebral atrophy Cerebral Atrophy Subdural Hemorrhage and multiple white matter White Matter The region of central nervous system that appears lighter in color than the other type, gray matter. It mainly consists of myelinated nerve fibers and contains few neuronal cell bodies or dendrites. Brown-Séquard Syndrome lesions on MRI
    • Delayed recognition

Differential Diagnosis

  • Alzheimer disease Alzheimer disease As the most common cause of dementia, Alzheimer disease affects not only many individuals but also their families. Alzheimer disease is a progressive neurodegenerative disease that causes brain atrophy and presents with a decline in memory, cognition, and social skills. Alzheimer Disease: progressive neurodegenerative disease that causes brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification atrophy Atrophy Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. Cellular Adaptation and presents with a decline in memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment, cognition, and social skills. Several genetic defects and risk factors have been described for Alzheimer disease Alzheimer disease As the most common cause of dementia, Alzheimer disease affects not only many individuals but also their families. Alzheimer disease is a progressive neurodegenerative disease that causes brain atrophy and presents with a decline in memory, cognition, and social skills. Alzheimer Disease, but there is no clear cause in the majority of cases. Diagnosis is based on clinical examination, neuropsychiatric testing, and imaging. There is no curative therapy, but symptomatic management with medications, including cholinesterase Cholinesterase Liver Function Tests inhibitors and others, may slow the progression.
  • Dementia with Lewy bodies Dementia with Lewy bodies A neurodegenerative disease characterized by dementia, mild parkinsonism, and fluctuations in attention and alertness. The neuropsychiatric manifestations tend to precede the onset of bradykinesia, muscle rigidity, and other extrapyramidal signs. Delusions and visual hallucinations are relatively frequent in this condition. Histologic examination reveals lewy bodies in the cerebral cortex and brain stem. Senile plaques and other pathologic features characteristic of alzheimer disease may also be present. Major Neurocognitive Disorders (DLB): Lewy bodies Lewy bodies Intracytoplasmic, eosinophilic, round to elongated inclusions found in vacuoles of injured or fragmented neurons. The presence of lewy bodies is the histological marker of the degenerative changes in lewy body disease and parkinson disease but they may be seen in other neurological conditions. They are typically found in the substantia nigra and locus coeruleus but they are also seen in the basal forebrain, hypothalamic nuclei, and neocortex. Parkinson’s Disease are thought to be responsible for impaired mental functions related to thinking, movement, behavior, and mood in DLB. The presence of Lewy bodies Lewy bodies Intracytoplasmic, eosinophilic, round to elongated inclusions found in vacuoles of injured or fragmented neurons. The presence of lewy bodies is the histological marker of the degenerative changes in lewy body disease and parkinson disease but they may be seen in other neurological conditions. They are typically found in the substantia nigra and locus coeruleus but they are also seen in the basal forebrain, hypothalamic nuclei, and neocortex. Parkinson’s Disease alters the level of neurotransmitters and neuromodulators in the brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification, principally dopamine Dopamine One of the catecholamine neurotransmitters in the brain. It is derived from tyrosine and is the precursor to norepinephrine and epinephrine. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. Receptors and Neurotransmitters of the CNS. Diagnosis is based on clinical symptoms and MRI. Management is symptomatic for specific manifestations of the disease.
  • Frontotemporal dementia Frontotemporal dementia Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of tau protein inclusions. Ftld includes three clinical syndromes: frontotemporal dementia, semantic dementia, and primary progressive nonfluent aphasia. Amyotrophic Lateral Sclerosis: neurodegenerative disorder characterized by loss of cognitive functions ( memory Memory Complex mental function having four distinct phases: (1) memorizing or learning, (2) retention, (3) recall, and (4) recognition. Clinically, it is usually subdivided into immediate, recent, and remote memory. Psychiatric Assessment, abstract thinking, reasoning Reasoning Decision-making Capacity and Legal Competence, and executive function) that severely impairs functioning and quality Quality Activities and programs intended to assure or improve the quality of care in either a defined medical setting or a program. The concept includes the assessment or evaluation of the quality of care; identification of problems or shortcomings in the delivery of care; designing activities to overcome these deficiencies; and follow-up monitoring to ensure effectiveness of corrective steps. Quality Measurement and Improvement of life. Clinical presentation includes behavioral changes, such as apathy Apathy Lack of emotion or emotional expression; a disorder of motivation that persists over time. Wernicke Encephalopathy and Korsakoff Syndrome. Diagnosis is based on interviewing caregivers/family members and imaging to rule out other pathology. Management is both pharmacologic and nonpharmacologic to lessen symptoms.
  • Parkinson’s disease: chronic, progressive neurodegenerative disorder. Although the cause of Parkinson’s disease is unknown, there are several genetic and environmental risk factors that are currently being studied. Individuals present clinically with resting tremor Resting Tremor Parkinson’s Disease, bradykinesia Bradykinesia Parkinson’s Disease, rigidity Rigidity Continuous involuntary sustained muscle contraction which is often a manifestation of basal ganglia diseases. When an affected muscle is passively stretched, the degree of resistance remains constant regardless of the rate at which the muscle is stretched. This feature helps to distinguish rigidity from muscle spasticity. Megacolon, and postural instability. Diagnosis is clinical. Treatment includes supportive physical and emotional care plus medications such as levodopa Levodopa The naturally occurring form of dihydroxyphenylalanine and the immediate precursor of dopamine. Unlike dopamine itself, it can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to dopamine. It is used for the treatment of parkinsonian disorders and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system. Parkinson’s Disease Drugs/carbidopa, monoamine oxidase Oxidase Neisseria type B inhibitors, and dopamine Dopamine One of the catecholamine neurotransmitters in the brain. It is derived from tyrosine and is the precursor to norepinephrine and epinephrine. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. Receptors and Neurotransmitters of the CNS agonists.
  • Atypical parkinsonian syndromes Atypical Parkinsonian Syndromes Atypical Parkinsonian syndromes are a group of neurodegenerative disorders characterized by Parkinsonian features although with different pathophysiology. These characteristics include progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD). Atypical Parkinsonian Syndromes: progressive supranuclear palsy Progressive Supranuclear Palsy A degenerative disease of the central nervous system characterized by balance difficulties; ocular motility disorders (supranuclear ophthalmoplegia); dysarthria; swallowing difficulties; and axial dystonia. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal mesencephalon; subthalamic nucleus; red nucleus; pallidum; dentate nucleus; and vestibular nuclei. Atypical Parkinsonian Syndromes ( PSP PSP A degenerative disease of the central nervous system characterized by balance difficulties; ocular motility disorders (supranuclear ophthalmoplegia); dysarthria; swallowing difficulties; and axial dystonia. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal mesencephalon; subthalamic nucleus; red nucleus; pallidum; dentate nucleus; and vestibular nuclei. Atypical Parkinsonian Syndromes) and corticobasal degeneration Corticobasal Degeneration Atypical Parkinsonian Syndromes ( CBD CBD Atypical Parkinsonian Syndromes) are neurodegenerative disorders characterized by their display of parkinsonian features but with different pathophysiology. Both PSP PSP A degenerative disease of the central nervous system characterized by balance difficulties; ocular motility disorders (supranuclear ophthalmoplegia); dysarthria; swallowing difficulties; and axial dystonia. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal mesencephalon; subthalamic nucleus; red nucleus; pallidum; dentate nucleus; and vestibular nuclei. Atypical Parkinsonian Syndromes and CBD CBD Atypical Parkinsonian Syndromes have cognitive manifestations and motor Motor Neurons which send impulses peripherally to activate muscles or secretory cells. Nervous System: Histology symptoms. Diagnosis is clinical and with MRI. Management is supportive and with levodopa Levodopa The naturally occurring form of dihydroxyphenylalanine and the immediate precursor of dopamine. Unlike dopamine itself, it can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to dopamine. It is used for the treatment of parkinsonian disorders and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system. Parkinson’s Disease Drugs/carbidopa for PSP PSP A degenerative disease of the central nervous system characterized by balance difficulties; ocular motility disorders (supranuclear ophthalmoplegia); dysarthria; swallowing difficulties; and axial dystonia. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal mesencephalon; subthalamic nucleus; red nucleus; pallidum; dentate nucleus; and vestibular nuclei. Atypical Parkinsonian Syndromes, but does not reduce symptoms in CBD CBD Atypical Parkinsonian Syndromes. Other medications may help with tremors, myoclonus Myoclonus Involuntary shock-like contractions, irregular in rhythm and amplitude, followed by relaxation, of a muscle or a group of muscles. This condition may be a feature of some central nervous system diseases; (e.g., epilepsy-myoclonic). Nocturnal myoclonus is the principal feature of the nocturnal myoclonus syndrome. Neurological Examination, and cognitive dysfunction.

References

  1. Das, J.M., Biagioni, M. C. (2021). Normal pressure hydrocephalus. StatPearls. Retrieved August 18, 2021, from http://www.ncbi.nlm.nih.gov/books/NBK542247/ 
  2. Berkowitz, A.L. (2016). Delirium, dementia, and rapidly progressive dementia. Chapter 22 of Clinical Neurology and Neuroanatomy: A Localization-based Approach. McGraw-Hill Education. http://accessmedicine.mhmedical.com/content.aspx?aid=1160205112 
  3. Graff-Radford, N.R. (2020). Normal pressure hydrocephalus. UpToDate. Retrieved August 18, 2021, from: https://www.uptodate.com/contents/normal-pressure-hydrocephalus 
  4. Sharma, R., Singh, G. (2020). Normal pressure hydrocephalus. Retrieved August 23, 2021, from https://radiopaedia.org/articles/normal-pressure-hydrocephalus?lang=us
  5. Williams, M.A., Malm, J. (2016). Diagnosis and treatment of idiopathic normal pressure hydrocephalus. Continuum 22:579–599. DOI: 10.1212/CON.0000000000000305

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