Disease

Definition

Episodic#

Jerboa Abbreviation

Optic neuritis

Optic neuritis is the general inflammation of the optical nerve

No

ON

Convulsion

Seizures are episodes of neuronal hyperactivity most commonly resulting in sudden, involuntary muscular contractions. They may also manifest as sensory disturbances, autonomic dysfunction and behavioral abnormalities, and impairment or loss of consciousness. Descriptions and classifications of seizures are complex and subject to change, because the etiology and pathogenesis of most seizures remain to be elucidated.

For the purpose of background incidence rates convulsion will be limited to generalized convulsions.

Yes

CONV

Anaphylaxis

Anaphylaxis is an acute hypersensitivity reaction with multi-organ-system involvement that can present as, or rapidly progress to, a severe life-threatening reaction. It may occur following exposure to allergens from a variety of sources including food, aeroallergens, insect venom, drugs, and immunizations.

For the purpose of background rates the definition will be limited to severe anaphylaxis involving multi-organ failure. In addition, we will differentiate between known (i.e. bee sting, drug, food etc., ANAK). and unknown (i.e. idiopathic, ANAI) precipitating triggers.

Yes

ANAI

ANAK

Encephalitis

encephalitis is defined as inflammation of the parenchyma of the brain. Strictly speaking, it is a pathologic diagnosis, in which the presence of inflammation, edema, and neuronophagia (neuronal cell death) is demonstrated by histopathology. This is generally associated with an infiltration of inflammatory cells and perivascular cuffing.

Myelitis, by corollary, is defined as inflammation of the parenchyma of the spinal cord. An inflammatory process involving both the brain and the spinal cord may be referred to as “encephalomyelitis”. Demonstration of this inflammation, regardless of the underlying cause, is considered confirmatory in the diagnosis of encephalitis/myelitis. A clinical diagnosis of encephalitis depends on the demonstration of brain dysfunction in the presence of evidence of an inflammatory process; this dysfunction may be cortical, subcortical (e.g., deep gray nuclei, brainstem), or both. Cerebral or cortical/subcortical dysfunction is generally manifested as (a) diffuse or global cortical dysfunction resulting in alteration of consciousness, impairment of mentation, or acute alteration of a person’s normal behavior, (b) focal cortical/subcortical dysfunction, resulting in so-called focal neurologic deficits, or (c) manifestations of both global dysfunction and focal deficits.

For the background rates we will differentiate between viral and other encephalitides.

No

ENCV(iral)

ENCO(other)

Vasculitis

The systemic vasculitides are an uncommon group of autoimmune diseases capable of causing multi organ failure and death. Clinical evaluation tools have been developed and provide a comprehensive assessment of patients. Serological markers, especially anti-neutrophil cytoplasm antibody (ANCA), pathology and imaging investigations are a useful addition, but are more valuable in diagnosis rather than monitoring of disease activity.

Primary vasculitides: Kawasaki disease; Behçet's disease; Polyarteritis nodosa or PAN; Wegener's granulomatosis; Cryoglobulinemia ; Takayasu's arteritis or pulseless disease; Churg-Strauss syndrome; Giant cell arteritis or temporal arteritis; Henoch-Schönlein purpura

Diagnosis: Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), anemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria.

Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of various organs involvement.

The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, and kidney. The biopsy elucidates the pattern of blood vessel inflammation.

An alternative to biopsy can be an angiogram (x-ray test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.

The background rates will be provided on total vasculitis.

No

VASC

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an acute polyneuropathy consisting of different subtypes. Acute inflammatory demyelinating polyradiculoneuropathy, the classic demyelinating form of GBS, accounts for 90% of all GBS cases in the Western world. Acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN) are axonal forms of GBS that are more prevalent in Asia, South and Central America, often preceded by infection by Campylobacter jejuni. AMAN and AMSAN may be mediated by specific anti-ganglioside antibodies that inhibit transient sodium ion (Na+) channels. Brighton Collaboration definition: James J. Sejvar, et al.. Guillain-D^Barre Syndrome: Case  definitions and guidelines for collection, analysis, and presentation of immunization safety data. (Manuscript)

Background rates will also include Miller Fisher syndrome

No

GBS

Demyelination

a degenerative process that erodes away the myelin sheath that normally protects nerve fibers. Demyelination exposes these fibers and appears to cause problems in nerve impulse conduction that may affect many physical systems. Demyelinization is seen in a number of diseases, particularly multiple sclerosis, transverse myelitis, Devic's disease, progressive multifocal leukoencephalopathy, Optic neuritis.

Optic neuritis and transverse myelitis are separate items and should not be counted as demyelinating disease, but separately

No

DMD

Bells Palsy

Bell's palsy is a paralysis of cranial nerve VII (the facial nerve) resulting in inability to control facial muscles on the affected side. Several conditions can cause a facial paralysis, e.g., brain tumor, stroke, and Lyme disease. However, if no specific cause can be identified, the condition is known as Bell's palsy. Bell's palsy is defined as an idiopathic unilateral facial nerve paralysis, usually self-limiting. The trademark is rapid onset of partial or complete palsy, usually in a single day.

Bell’s palsy is largely a diagnosis of exclusion, but certain features in the history and physical examination help distinguish it from facial paralysis due to other conditions: eg, abrupt onset with complete, unilateral facial weakness at 24 to 72 hours, and, on the affected side, numbness or pain around the ear, a reduction in taste, and hypersensitivity to sounds. Corticosteroids and antivirals given within 10 days of onset have been shown to help. But Bell palsy resolves spontaneously without treatment in most patients within 6 months.

Brighton Collaboration definition: Rath B, et al. Bells palsy: Case definitions and guidelines for collection, analysis, and presentation of immunization safety data. (Manuscript)

No

BP

Thrombocytopenia

thrombocytopenia is defined as a platelet count below 150 × 10⁹ L⁻¹ confirmed by bloodsmear or the presence of clinical signs and symptoms of spontaneous bleeding. Presentations of spontaneous (i.e., non-traumatic) bleeding include purpura (i.e., petechiae, purpura sensu stricto, ecchymosis), hemorrhagic oozing of skin lesions including rashes, hematoma, bruising, hematemesis, hematochezia, occult bleeding per rectum, epistaxis, hemoptysis, hematuria, vaginal bleeding other than menstruation, conjunctival bleeding, intracranial bleeding. Includes Idiopathic thrombocytopenic purpura, idiopathic thrombopenia, ITP

For the background rates we will include the wide definition but differentiate between hospitalization for thrombocytopena (primary diagnosis only) and outpatient diagnosis.

Yes

THROM

Transverse myelitis

Transverse myelitis is a neurological disorder caused by an inflammatory process of the grey and white matter of the spinal cord, and can cause axonal demyelination.

(http://en.wikipedia.org/wiki/Transverse_myelitis; http://www.ninds.nih.gov/disorders/transversemyelitis/detail_transversemyelitis.htm).

In the background rate calculation we will differentiate between viral and other transverse myelitis.

No

TM

Autoimmune hepatitis

A chronic disease of unknown cause, characterized by continuing hepatocellular inflammation and necrosis, which tends to progress to cirrhosis. Autoimmune chronic active hepatitis is a chronic necroinflammatory hepatitis of unknown etiology, characterized histologically by a dense mononuclear infiltrate in the portal tracts and serologically by antibodies against liver-specific and non-liver-specific antigens and increased immunoglobulin G (IgG) levels. Autoimmune hepatitis may present as acute hepatitis, chronic hepatitis or well-established cirrhosis. Some cases of drug-induced liver disease may have an immune-mediated basis. The clinical picture may share features with acute viral hepatitis and can be distinguished from these upon testing for ASMA and ANA and possibly liver biopsy. (http://emedicine.medscape.com/article/172356-overview)

No

AIH

Spontaneous abortion

the expulsion of an embryo or fetus before the 22nd week of gestation. Including miscarriages and premature birth. Premature or preterm birth refers to the birth of a baby of less than 37 weeks gestational age.

Background rates will not be calculated now (but obtained from other sources)

Yes

ABORT

Sudden unexpected death

sudden cardiac death refers to: natural death from cardiac causes, heralded by abrupt loss of consciousness within one hour of the onset of acute symptoms.

Sudden infant death refers to: sudden unexpected death of an infant <=2 year of age, with onset of the fatal episode apparently occurring during sleep that remains unexplained after a thorough investigation, including performance of a complete autopsy and review of the circumstances of death and the clinical history.

Background rates of sudden unexpected death will not be limited to age groups but will only be done in databases in which the cause of death can be assessed.

No

SUD