icd 10 code for susac syndrome

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What is Susac syndrome?

This is a rare disease of unknown origin, most likely an autoimmune endotheliopathy, causing an arteriolar microangiopathy of the brain, cochlea, and retina. Susac syndrome tends to affect young women, but it can also occur in men.

Which radiographic findings are characteristic of Susac's syndrome?

Radiographic appearance. Multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) can mimic the MRI changes seen in patients with Susac's syndrome. However, the callosal lesions in Susac's syndrome are centrally located. In comparison, patients with MS and ADEM typically have lesions involving the undersurface of the corpus callosum.

What is the difference between Susac's syndrome and multiple sclerosis (MS)?

However, the callosal lesions in Susac's syndrome are centrally located. In comparison, patients with MS and ADEM typically have lesions involving the undersurface of the corpus callosum. Deep gray matter involvement commonly occurs in ADEM but is very rare in MS. Leptomeningeal involvement is not typical of either MS or ADEM.

What is the ICD 10 code for circulatory system disease?

Diseases of the circulatory system complicating pregnancy, unspecified trimester. O99.419 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2019 edition of ICD-10-CM O99.419 became effective on October 1, 2018.

What is Susac disease?

Susac syndrome is a relatively rare disorder characterized by three main problems: impaired brain function (encephalopathy), partial or complete blockage (occlusion) of the small arteries and capillaries that supply blood to the retina (branch retinal artery occlusion, or BRAO), and inner ear disease (hearing loss, ...

How many cases of Susac syndrome are there?

Susac syndrome (SuS) prevalence is still unknown. To date more than 500 cases have been reported worldwide. Young females (20-40 years) are more affected (female: male ratio 3.5:1). The age at onset ranges from 8 to 72 years (mean age: 32 years).

What is the ICD-10 code for encephalopathy unspecified?

ICD-10 code G93. 40 for Encephalopathy, unspecified is a medical classification as listed by WHO under the range - Diseases of the nervous system .

What is multifactorial encephalopathy?

Encephalopathy means disorder or disease of the brain. Multifactorial means multiple factors. According to the definition, “multifactorial encephalopathy does not refer to a single disease, but rather to a syndrome of global brain dysfunctions; this syndrome can be caused by many different illnesses.”

What is Marchiafava Bignami disease?

Marchiafava-Bignami disease (MBD) is a very rare disorder of demyelination/necrosis of the corpus callosum and the near subcortical white matter that is especially predominant in ill-fed alcoholics[1]. It was discovered in 1903 by Italian pathologists Ettore Marchiafava and Amico Bignami.

What autoimmune causes ringing in ears?

Autoimmune inner ear disease (AIED), is a rare disease that happens when your body's immune system mistakenly attacks your inner ear. It can cause dizziness, ringing in your ears, and hearing loss. Less than 1% of the 28 million Americans who have hearing loss have it because of AIED.

What is the ICD-10 code for G93 41?

ICD-10 | Metabolic encephalopathy (G93. 41)

What is the ICD-10 code for G93 40?

Encephalopathy, unspecified G93.

What is the ICD-10 code for altered mental status?

R41. 82 altered mental status, unspecified.

What is Wernicke's encephalopathy?

Wernicke syndrome, also known as Wernicke encephalopathy, is a neurological disease characterized by three main clinical symptoms: confusion, the inability to coordinate voluntary movement (ataxia) and eye (ocular) abnormalities.

What is the difference between encephalitis and encephalopathy?

The words sound similar, but they are different conditions. In encephalitis, the brain itself is swollen or inflamed. Encephalopathy, on the other hand, refers to the mental state that can happen because of several types of health problems. But encephalitis can cause encephalopathy.

What are the different types of encephalopathy?

The following are some major types of encephalopathy, along with their causes.Chronic traumatic encephalopathy. ... Glycine encephalopathy. ... Hashimoto's encephalopathy. ... Hepatic encephalopathy. ... Hypertensive encephalopathy. ... Hypoxic ischemic encephalopathy. ... Toxic-metabolic encephalopathy. ... Infectious encephalopathies.More items...

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Epidemiology

Susac syndrome (SuS) prevalence is still unknown. To date more than 500 cases have been reported worldwide. Young females (20-40 years) are more affected (female: male ratio 3.5:1). The age at onset ranges from 8 to 72 years (mean age: 32 years).

Clinical description

Characteristic is a triad of encephalopathy (cognitive and behavioral disturbances, personality changes, psychosis, preceding headaches) and/or focal CNS dysfunction, visual dysfunction due to BRAO and SNHL. The components of the triad may not be concomitantly present and may develop successively.

Etiology

The etiology and precise pathophysiology are not yet clear. According to recent data, clonally expanding autoreactive cytotoxic CD8+ T cells cause inflammation-mediated injury of the microvascular endothelium which leads to swelling of endothelial cells, vessel occlusion in the affected organ and finally micro-ischaemic damage and dysfunction.

Diagnostic methods

The diagnosis is based on the demonstration of the typical triad. There is no singular ''marker'' of SuS. Beside the clinical examination, most relevant diagnostic tools are brain magnetic resonance imaging (MRI), retinal fluorescein angiography (FA), and audiometry.

Differential diagnosis

Inflammatory demyelinating CNS disease (such as multiple sclerosis, acute disseminated encephalitis, neuromyelitis optica spectrum disorders), autoimmune encephalitis, and various other diseases involving CNS, retina, and inner ear (including infections, malignancies, psychotic disorders, cerebrovascular disease, migraine, Meniere disease, isolated BRAO) and a variety of autoimmune diseases such as Cogan syndrome, Eales disease, autoimmune inner-ear disease, polyarteritis nodosa, Wegener granulomatosis, Churg-Strauss syndrome, systemic lupus erythematosus, antiphospholipid syndrome, Sjögren syndrome and Behçet disease..

Management and treatment

No evidence-based treatment strategies exist. Empirically, pulsed glucocorticosteroid treatment is effective in the acute phase. In severe cases treatment may be complemented by cyclophosphamide and/or intravenous immunoglobulins (ivIG), methotrexate (MTX), azathioprine (AZA) or mycophenolate mofetil (MMF).

How old is Susac syndrome?

Susac syndrome affects women more than men. The age at which symptoms begin is usually between 20 and 40 years, but some people have symptoms earlier or later than this age range. [1] The cause of Susac syndrome is still unknown.

What is the treatment for Susac syndrome?

[2] . Treatment options include corticosteroids and cyclophosphamide as well as anticoagulation medications. Most people recover from Susac syndrome with treatment. [1]

How long does Susac last?

Other people have symptoms for a couple of episodes, after which time the symptoms go away. Finally, some people have a chronic, relapsing course with frequent flare-ups and remissions. [6] The condition typically lasts from 2 to 4 years, but may be shorter or longer depending on the person.

Do people with susac syndrome have vertigo?

The ears also have a role in regulating our balance. Therefore, some people with Susac syndrome experience vertigo and feel like their surroundings are spinning. [1] People with Susac syndrome may find that their symptoms go away and return again over a period of years.

Can Susac cause hearing loss?

This can cause dark spots in the vision, but some people have no symptoms of BRAO. [1] Susac syndrome can also cause hearing loss due to poor circulation to the ears.

Can Susac syndrome cause headaches?

However, all three body parts are not affected in all people with Susac syndrome. [1] A headache is often one of the earliest symptoms of Susac syndrome. Recurrent headaches may be a sign of encephalopathy, or brain disease.

Is Susac syndrome inherited?

To our knowledge, there is no evidence that Susac syndrome is inherited, and no familial cases of Susac syndrome have been reported. Cases described in the medical literature have occurred in people with no known history of the condition in their family.

What is the ICD10 code for Susacs syndrome? And the ICD9 code for Susacs syndrome?

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What is the history of Susacs syndrome?

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What is O99 in medical terms?

O99- Other maternal diseases classifiable elsewhere but complicating pregnancy, childbirth and the puerperium

When will the ICD-10-CM O99.419 be released?

The 2022 edition of ICD-10-CM O99.419 became effective on October 1, 2021.

What is the O99.353?

O99.353 Diseases of the nervous system complicating pregnancy, third trimester. O99.354 Diseases of the nervous system complicating childbirth. O99.355 Diseases of the nervous system complicating the puerperium. O99.4 Diseases of the circulatory system complicating pregnancy, childbirth and the puerperium.

What is Susac's syndrome?

Neurology. Susac's syndrome ( retinocochleocerebral vasculopathy) is a very rare form of microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss . The cause is unknown but the current thinking is that antibodies are produced against endothelial cells in tiny arteries which leads to damage and ...

Who was the first person to describe Susac syndrome?

William F. Hoyt was the first to call the syndrome "Susac syndrome" and later Robert Daroff asked Dr. Susac to write an editorial in Neurology about the disorder and to use the eponym of Susac syndrome in the title, forever linking this disease with him.

Can Susac's syndrome mimic MS?

A concern about this illness is that it mimics multiple sclerosis when looking at the vision loss and brain lesions. If close attention is not paid to the retina of a patient with vision loss and brain lesions, their symptoms may be mistaken for MS instead of Susac's syndrome. This may account for the low prevalence of the illness. There is also a pathological similarity between the endotheliopathy in Susac's syndrome with that seen in juvenile dermatomyositis .

Is demyelination a sign of Susac's syndrome?

Demyelination is not a typical feature of Susac's syndrome. Muscle biopsies from such patients are usually normal, but some have also shown nonspecific signs of inflammation such as dense hyaline material surrounding endomysial capillaries.

Is demyelination a characteristic feature of Susac's syndrome?

Demyelination is not a typical feature of Susac's syndrome.