Neuropathology

Last updated
Micrograph of a vasculitic neuropathy. Plastic embedded. Toluidine blue stain. Vasculitic neuropathy - plastics - low mag.jpg
Micrograph of a vasculitic neuropathy. Plastic embedded. Toluidine blue stain.

Neuropathology is the study of disease of nervous system tissue, usually in the form of either small surgical biopsies or whole-body autopsies. Neuropathologists usually work in a department of anatomic pathology, but work closely with the clinical disciplines of neurology, and neurosurgery, which often depend on neuropathology for a diagnosis. Neuropathology also relates to forensic pathology because brain disease or brain injury can be related to cause of death. Neuropathology should not be confused with neuropathy, which refers to disorders of the nerves themselves (usually in the peripheral nervous system) rather than the tissues. In neuropathology, the branches of the specializations of nervous system as well as the tissues come together into one field of study.

Contents

Methodology

The work of the neuropathologist consists largely of examining autopsy or biopsy tissue from the brain and spinal cord to aid in diagnosis of disease. [1] Tissues are also observed through the eyes, muscles, surfaces of organs, and tumors. The biopsy is usually requested after a mass is detected by radiologic imaging, the imaging in turn driven by presenting signs and symptoms of a patient. CT scans are also used to discover issues in the patient. As for autopsies, the principal work of the neuropathologist is to help in the post-mortem diagnosis of various forms of dementia and other conditions that affect the central nervous system. Tissue samples are researched within the lab for diagnosis as well as forensic investigations.

Biopsies can also consist of the skin. Epidermal nerve fiber density testing (ENFD) is a more recently developed neuropathology test in which a punch skin biopsy is taken to identify small fiber neuropathies by analyzing the nerve fibers of the skin. This pathology test is becoming available in select labs as well as many universities; it replaces the traditional sural nerve biopsy test as less invasive. It is used to identify painful small fiber neuropathies.

Neuropathologists work in hospital labs and clinics, universities, or with the government depending on the situation. They often do not work with patients but only with medical professionals or other officials behind the scene. They research using information given to them by other neurologists and/ or physicians. Neuropathologists may also research in coroner's or morgue offices for forensic projects. The ultimate goal of neuropathologists is to find the medical issue and then formulate a timeline in which to cure the patient's tissue.

Focus of specialization

In many English-speaking countries neuropathology is considered a subfield of anatomical pathology. In contrast, there are a number of independent university chairs in neuropathology and even institutes of neuropathology in German-speaking countries due to a different historical background. A physician who specializes in neuropathology, usually by completing a fellowship after a residency in anatomical or general pathology, is called a neuropathologist. In day-to-day clinical practice, a neuropathologist is a consultant for other physicians. If a disease of the nervous system is suspected, and the diagnosis cannot be made by less invasive methods, a biopsy of nervous tissue is taken and sent to the neuropathologist, who examines it using a microscope or certain molecular methods to make a definitive diagnosis.

Many neuropathologists in Europe have a background in the clinical neurosciences (neurology, psychiatry) as well as pathology.

In the US system

Neuropathologists are physicians with a Doctor of Medicine (MD) or Doctor of Osteopathic Medicine (DO) degree. They must finish either 3 or 4 years of an anatomical pathology residency followed by 2 years of a neuropathology fellowship and be certified by the American Board of Pathology [2] in both anatomical and neuropathology. This is less specialized neuropathology training than in most other countries. It is also quite common for neuropathologists to have a Ph.D. in a related field. Neuropathologists must have strong communication abilities as they must analyze results and be able to explain the results to patients and/ or physicians (in paper or verbally).

In the UK/Canadian/Commonwealth system

Neuropathologists are medically qualified practitioners who are registered with the General Medical Council in the UK. A postgraduate qualification in neuropathology is obtained through training and an examination overseen by the Royal College of Pathologists UK. A neuropathologist has training in anatomic pathology followed by training in relation to diagnosis of diseases of the nervous system and muscle. The training in other European and commonwealth countries is similar. In Canada, Neuropathologists complete a 5-year Royal College of Physicians and Surgeons of Canada Neuropathology residency including a year of clinical medicine and a year of anatomical pathology. It is quite common for neuropathologists to have PhDs in a related field.

In addition to examining central nervous system tissue, the neuropathologist usually is assigned the task of examining muscle and peripheral nerve biopsies. Muscle biopsies are taken to aid in the diagnosis of muscle diseases (such as polymyositis, mitochondrial myopathy, etc.). Peripheral nerve is assessed to help work up patients with suspected peripheral neuropathies secondary to such conditions as vasculitis and amyloidosis.

Neuropathology is a heavily research-oriented field.

Prominent historical and current figures

Santiago Ramon y Cajal is considered one of the founders of modern neuroanatomy. Alois Alzheimer, the person after whom Alzheimer's disease is named, is considered an important early contributor to the field.

There are many neuropathologists around the world who have made important clinical and research contributions toward our understanding of diseases that specifically affect the brain (degenerative diseases, multiple sclerosis, stroke, brain tumors, trauma and neuromuscular diseases). The majority are members of the International Society of Neuropathology (ISN). For neuropathologists practicing within the United States of America please refer to the Membership Directory available through the American Association of Neuropathologists (AANP) website. There are also Membership Directories available for many of the neuropathology societies that exist in other specific countries and/or regions of the world (British, European, Canadian... etc.).

Progress

A European Board Examination in Neuropathology which emphasizes the importance of proper training in the neurosciences is currently being established (www.euro-cns.org). The most recent international congress of neuropathology was held in Tokyo, Japan, in September 2018.

Journals

Academic neuropathology is served by several specialist neuropathology journals. Acta Neuropathologica is the neuropathology journal with the highest impact factor. [3] Some journals are sponsored by national or international neuropathology associations: Brain Pathology is the official journal of the International Society of Neuropathology, Neuropathology & Applied Neurobiology is sponsored by the British Neuropathological Society, the Journal of Neuropathology & Experimental Neurology is the official journal of the American Association of Neuropathologists (AANP) and "Neuropathology" is the official journal of the Japanese Society of Neuropathology.

Related Research Articles

<span class="mw-page-title-main">Neurosurgery</span> Medical specialty of disorders which affect any portion of the nervous system

Neurosurgery or neurological surgery, known in common parlance as brain surgery, is the medical specialty concerned with the surgical treatment of disorders which affect any portion of the nervous system including the brain, spinal cord and peripheral nervous system.

<span class="mw-page-title-main">Pathology</span> Study of the causes and effects of disease or injury, and how they arise

Pathology is the study of disease and injury. The word pathology also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in the context of modern medical treatment, the term is often used in a narrower fashion to refer to processes and tests that fall within the contemporary medical field of "general pathology", an area which includes a number of distinct but inter-related medical specialties that diagnose disease, mostly through analysis of tissue and human cell samples. Idiomatically, "a pathology" may also refer to the predicted or actual progression of particular diseases, and the affix pathy is sometimes used to indicate a state of disease in cases of both physical ailment and psychological conditions. A physician practicing pathology is called a pathologist.

<span class="mw-page-title-main">Anatomical pathology</span> Medical specialty

Anatomical pathology (Commonwealth) or anatomic pathology (U.S.) is a medical specialty that is concerned with the diagnosis of disease based on the macroscopic, microscopic, biochemical, immunologic and molecular examination of organs and tissues. Over the 20th century, surgical pathology has evolved tremendously: from historical examination of whole bodies (autopsy) to a more modernized practice, centered on the diagnosis and prognosis of cancer to guide treatment decision-making in oncology. Its modern founder was the Italian scientist Giovan Battista Morgagni from Forlì.

<span class="mw-page-title-main">Optic nerve</span> Second cranial nerve, which connects the eyes to the brain

In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual information from the retina to the brain. In humans, the optic nerve is derived from optic stalks during the seventh week of development and is composed of retinal ganglion cell axons and glial cells; it extends from the optic disc to the optic chiasma and continues as the optic tract to the lateral geniculate nucleus, pretectal nuclei, and superior colliculus.

Diabetic neuropathy is various types of nerve damage associated with diabetes mellitus. Symptoms depend on the site of nerve damage and can include motor changes such as weakness; sensory symptoms such as numbness, tingling, or pain; or autonomic changes such as urinary symptoms. These changes are thought to result from a microvascular injury involving small blood vessels that supply nerves. Relatively common conditions which may be associated with diabetic neuropathy include distal symmetric polyneuropathy; third, fourth, or sixth cranial nerve palsy; mononeuropathy; mononeuropathy multiplex; diabetic amyotrophy; and autonomic neuropathy.

<span class="mw-page-title-main">Peripheral neuropathy</span> Nervous system disease affecting nerves beyond the brain and spinal cord

Peripheral neuropathy, often shortened to neuropathy, is a general term describing damage or disease affecting the nerves. Damage to nerves may impair sensation, movement, gland, or organ function depending on which nerves are affected; in other words, neuropathy affecting motor, sensory, or autonomic nerves result in different symptoms. More than one type of nerve may be affected simultaneously. Peripheral neuropathy may be acute or chronic, and may be reversible or permanent.

<span class="mw-page-title-main">Polyneuropathy</span> Medical condition

Polyneuropathy is damage or disease affecting peripheral nerves in roughly the same areas on both sides of the body, featuring weakness, numbness, and burning pain. It usually begins in the hands and feet and may progress to the arms and legs and sometimes to other parts of the body where it may affect the autonomic nervous system. It may be acute or chronic. A number of different disorders may cause polyneuropathy, including diabetes and some types of Guillain–Barré syndrome.

Neurapraxia is a disorder of the peripheral nervous system in which there is a temporary loss of motor and sensory function due to blockage of nerve conduction, usually lasting an average of six to eight weeks before full recovery. Neurapraxia is derived from the word apraxia, meaning “loss or impairment of the ability to execute complex coordinated movements without muscular or sensory impairment”.

<span class="mw-page-title-main">Nerve conduction study</span> Diagnostic test for nerve function

A nerve conduction study (NCS) is a medical diagnostic test commonly used to evaluate the function, especially the ability of electrical conduction, of the motor and sensory nerves of the human body. These tests may be performed by medical specialists such as clinical neurophysiologists, physical therapists, physiatrists, and neurologists who subspecialize in electrodiagnostic medicine. In the United States, neurologists and physiatrists receive training in electrodiagnostic medicine as part of residency training and in some cases acquire additional expertise during a fellowship in clinical neurophysiology, electrodiagnostic medicine, or neuromuscular medicine. Outside the US, clinical neurophysiologists learn needle EMG and NCS testing.

<span class="mw-page-title-main">Neuritis</span> Inflammation of a nerve or generally any part of the nervous system

Neuritis is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired transmission of neural signals and leads to aberrant nerve function. Neuritis is often conflated with neuropathy, a broad term describing any disease process which affects the peripheral nervous system. However, neuropathies may be due to either inflammatory or non-inflammatory causes, and the term encompasses any form of damage, degeneration, or dysfunction, while neuritis refers specifically to the inflammatory process.

<span class="mw-page-title-main">Sural nerve</span> Pure cutaneous nerve of the posterolateral leg to the lateral ankle

The sural nerve(L4-S1) is generally considered a pure cutaneous nerve of the posterolateral leg to the lateral ankle. The sural nerve originates from a combination of either the sural communicating branch and medial sural cutaneous nerve, or the lateral sural cutaneous nerve. This group of nerves is termed the sural nerve complex. There are eight documented variations of the sural nerve complex. Once formed the sural nerve takes its course midline posterior to posterolateral around the lateral malleolus. The sural nerve terminates as the lateral dorsal cutaneous nerve.

<span class="mw-page-title-main">Chronic inflammatory demyelinating polyneuropathy</span> Medical condition

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy. CIDP is closely related to Guillain–Barré syndrome and it is considered the chronic counterpart of that acute disease. Its symptoms are also similar to progressive inflammatory neuropathy. It is one of several types of neuropathy.

<span class="mw-page-title-main">Familial amyloid polyneuropathy</span> Medical condition

Familial amyloid polyneuropathy, also called transthyretin-related hereditary amyloidosis, transthyretin amyloidosis abbreviated also as ATTR, or Corino de Andrade's disease, is an autosomal dominant neurodegenerative disease. It is a form of amyloidosis, and was first identified and described by Portuguese neurologist Mário Corino da Costa Andrade, in 1952. FAP is distinct from senile systemic amyloidosis (SSA), which is not inherited, and which was determined to be the primary cause of death for 70% of supercentenarians who have been autopsied. FAP can be ameliorated by liver transplantation.

Small fiber peripheral neuropathy is a type of peripheral neuropathy that occurs from damage to the small unmyelinated and myelinated peripheral nerve fibers. These fibers, categorized as C fibers and small Aδ fibers, are present in skin, peripheral nerves, and organs. The role of these nerves is to innervate some skin sensations and help control autonomic function. It is estimated that 15–20 million people in the United States have some form of peripheral neuropathy.

Hereditary sensory and autonomic neuropathy (HSAN) or hereditary sensory neuropathy (HSN) is a condition used to describe any of the types of this disease which inhibit sensation.

<span class="mw-page-title-main">Nerve compression syndrome</span> Human disease

Nerve compression syndrome, or compression neuropathy, or nerve entrapment syndrome, is a medical condition caused by chronic, direct pressure on a peripheral nerve. It is known colloquially as a trapped nerve, though this may also refer to nerve root compression. Its symptoms include pain, tingling, numbness and muscle weakness. The symptoms affect just one particular part of the body, depending on which nerve is affected. The diagnosis is largely clinical and can be confirmed with diagnostic nerve blocks. Occasionally imaging and electrophysiology studies aid in the diagnosis. Timely diagnosis is important as untreated chronic nerve compression may cause permanent damage. A surgical nerve decompression can relieve pressure on the nerve but cannot always reverse the physiological changes that occurred before treatment. Nerve injury by a single episode of physical trauma is in one sense an acute compression neuropathy but is not usually included under this heading, as chronic compression takes a unique pathophysiological course.

Electrodiagnosis (EDX) is a method of medical diagnosis that obtains information about diseases by passively recording the electrical activity of body parts or by measuring their response to external electrical stimuli. The most widely used methods of recording spontaneous electrical activity are various forms of electrodiagnostic testing (electrography) such as electrocardiography (ECG), electroencephalography (EEG), and electromyography (EMG). Electrodiagnostic medicine is a medical subspecialty of neurology, clinical neurophysiology, cardiology, and physical medicine and rehabilitation. Electrodiagnostic physicians apply electrophysiologic techniques, including needle electromyography and nerve conduction studies to diagnose, evaluate, and treat people with impairments of the neurologic, neuromuscular, and/or muscular systems. The provision of a quality electrodiagnostic medical evaluation requires extensive scientific knowledge that includes anatomy and physiology of the peripheral nerves and muscles, the physics and biology of the electrical signals generated by muscle and nerve, the instrumentation used to process these signals, and techniques for clinical evaluation of diseases of the peripheral nerves and sensory pathways.

<span class="mw-page-title-main">Andermann syndrome</span> Medical condition

Andermann syndrome, also known as agenesis of corpus callosum with neuronopathy (ACCPN) and Charlevoix disease, among other names, is a very rare neurodegenerative genetic disorder that damages the nerves used to control muscles and related to sensation and is often associated with agenesis of the corpus collosum.

Peripheral mononeuropathy is a nerve related disease where a single nerve, that is used to transport messages from the brain to the peripheral body, is diseased or damaged. Peripheral neuropathy is a general term that indicates any disorder of the peripheral nervous system. The name of the disorder itself can be broken down in order to understand this better; peripheral: in regard to peripheral neuropathy, refers to outside of the brain and spinal cord; neuro: means nerve related; -pathy; means disease. Peripheral mononeuropathy is a disorder that links to Peripheral Neuropathy, as it only effects a single peripheral nerve rather than several damaged or diseased nerves throughout the body. Healthy peripheral nerves are able to “carry messages from the brain and spinal cord to muscles, organs, and other body tissues”.

Vasculitic neuropathy is a peripheral neuropathic disease. In a vasculitic neuropathy there is damage to the vessels that supply blood to the nerves. It can be as part of a systemic problem or can exist as a single-organ issue only affecting the peripheral nervous system (PNS). It is diagnosed with the use of electrophysiological testing, blood tests, nerve biopsy and clinical examination. It is a serious medical condition that can cause prolonged morbidity and disability and generally requires treatment. Treatment depends on the type but it is mostly with corticosteroids or immunomodulating therapies.

References

  1. "Neuropathology". UPMC. Retrieved 4 October 2020.
  2. elevationweb.org. "American Board of Pathology". www.abpath.org. Archived from the original on 22 March 2018. Retrieved 9 April 2018.
  3. "Acta Neuropathologica". springer.com. Archived from the original on 9 April 2018. Retrieved 9 April 2018.

Further reading