Proposed Research Criteria for Corticobasal Degeneration

Reference Inclusion criteria Exclusion criteria Lang et al., 1994 Rigidity plus one cortical sign (apraxia, cortical sensory loss, or alien limb). Or Asymmetric rigidity, dystonia and focal reflex myoclonus. Early dementia, early vertical gaze palsy, rest tremor, severe autonomic disturbances, sustained responsiveness to levodopa, lesions on imaging studies indicating another pathological condition. From Current Clinical Neurology Diagnostic Criteria in Neurology Edited by A. J. Lerner Humana...

Intracranial hemorrhage or vascular occlusion 10 Mononeuropathy singlemultiplex

Disturbed function of one or more peripheral nerve(s) resulting in weakness paralysis or sensory dysfunction because of either conduction block in motor nerve fibers or axonal loss. Conduction block is related to demyelination with preservation of axon continuity. Remyelination may be rapid and complete. If axonal interruption takes place, axonal degeneration occurs below the site of interruption and recovery is often slow and incomplete. Sensory symptoms and sensory loss may affect all...

External Compression Headache

International Headache Society Diagnostic Criteria for External Compression Headache A. Results from the application of external pressure in the forehead or the scalp. B. Is felt in the area subjected to pressure. D. Is prevented by avoiding the precipitating cause. E. Is not associated with organic cranial or intracranial disease. Adapted with permission from Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders, 2nd...

Diagnostic Criteria According to the International Headache Society for Migraine With Aura

At least two headache attacks by history, consistent with criteria 2-7. 2. No other disease present that might cause headache or neurological visual changes. 3. Fully reversible visual or sensory or speech symptoms, and no motor weakness. 4. Unilateral headache location. 5. At least one symptom that develops gradually (> 5 minutes) or a variety of symptoms occurring in a sequence. 6. Various symptoms lasting between 5 and 60 minutes. 7. Headache also meets criteria for migraine without aura,...

Chronic paroxysmal hemicrania

At least 30 attacks fulfilling criteria B-E. B. Attacks of severe unilateral orbital, supraorbital, and or temporal pain always on the same side, lasting 2-45 minutes. C. Attack frequency greater than five a day for more than half the time and tearing (periods with lower frequency may occur). D. Pain is associated with at least one of the following signs symptoms on the pain side E. At least one of the following 1. There is no suggestion of one of the disorders such as trigeminal neuralgia,...

Cervicogenic Headache

Headaches caused by disorders of the neck has been a controversial subject some feel that it is, at best, a rare syndrome. The original diagnostic criteria were proposed by Sjaastad in 1990, and are shown in Table 1. In a comprehensive review by Antonacci et al., the sensitivity and specificity of the original criteria were measured in an Italian clinic population. Their groups (labeled A, B, and C) correspond to individuals with varying symptoms. Group A includes those individuals with the...

International Headache Society Diagnostic Criteria for Cluster Headache

At least five attacks fulfilling criteria B-D. B. Severe or very severe unilateral orbital, supraorbital, and or temporal pain lasting 15-180 minutes if untreated.3 C. Headache is accompanied by at least one of the following 1. Ipsilateral conjunctival injection and or lacrimation. 2. Ipsilateral nasal congestion and or rhinorrhea. 4. Ipsilateral forehead and facial sweating. 5. Ipsilateral miosis and or ptosis. 6. A sense of restlessness or agitation. D. Attacks have a frequency from one...

Diagnostic Criteria for Hemicrania Continua

Headache for a total of more than 2 months. 1. Unilaterality without side shift.3 2. Absolute and protracted indomethacin effect.* 3. Long-lasting, repetitive attacks hours days weeks, with a tendency to a fluctuating chronic pattern over time.c 4. Intensity of pain mild, moderate or severe (not excruciatingly severe). C. Other nonobligatory but frequent characteristics of the pain syndrome 1. Female sex. 1. Relative shortage of local autonomic phenomena.d 2. Relative lack of migraine...

Acute confusional state see Delirium section in Chapter

Disturbance of consciousness or level of arousal characterized by reduced ability to focus, maintain, or shift attention, and accompanied by disturbances of cognition, mood, affect, and or behavior. The disturbances typically develop over hours to days and tend to fluctuate during the course of the day. They include hypo- and hyperaroused states and encompass the spectrum from delirium to coma. Diagnostic criteria Disturbance of consciousness or level of arousal with reduced ability to focus,...

Diagnostic Criteria for Sunct Syndrome

SUNCT (short-lasting unilateral neuralgiform headache with conjunctival injection and (earing) syndrome is characterized by short-lasting attacks of unilateral pain that are much briefer than those seen in any other trigeminal-autonomic cephalagia and very often accompanied by prominent lacrimation and redness of the ipsilateral eye. A. At least 20 attacks fulfilling criteria B-D. B. Attacks of unilateral orbital, supraorbital, or temporal stabbing or pulsating pain lasting 5-240 seconds. C....

Chronic Fatigue Syndrome

The criteria for diagnosing chronic fatigue syndrome were officially defined by the Centers for Disease Control in 1988 and revised in 2001. The Oxford criteria differ slightly. The British criteria insist on the presence of mental fatigue, although the American criteria include a requirement for several physical symptoms, reflecting the belief that chronic fatigue syndrome has an underlying immune or infectious pathology. There may be overlaps between individuals satisfying diagnostic criteria...

Hemicrania Continua And Paroxysmal Hemicranias

Originally described by Sjaastad and Spierings in 1984, hemicrania continua is a primary headache syndrome classified among the indomethacin-responsive headaches because of its selective and consistent response to this agent. It shares some features with cluster headache and migraines as well. Other hemicranial headaches share clinical and probably a pathophysiological basis, and are grouped here. The autonomic manifestations of these disorders are a common feature, and should be routinely

Normal Pressure Hydrocephalus

Since its first descriptions in the early 1960s, normal pressure hydrocephalus (NPH) has been difficult to recognize, and conclusive diagnosis relied on response to cerebrospinal fluid shunting. The clinical manifestations classically consist of the triad of gait apraxia, urinary incontinence, and dementia. Neuroimaging has made diagnosis of enlarged ventricles relatively easy, although distinguishing ventriculomegaly secondary to brain atrophy vs idiopathic NPH remains challenging. A variety...

Facioscapulohumeral Muscular Dystrophy

Facioscapulohumeral muscular dystrophy (FSHD) is a dominantly inherited muscular dystrophy affecting about 1 in 20,000 individuals. The weakness is not restricted to areas named in the disease name weakness may occur in hip girdle, ankle dorsiflexors, and occasionally oropharynx. Extramuscular manifestations of this slowly progressing myopathy may include hearing loss and retinal vasculopathy. All patients with a confirmed diagnosis of FSHD carry a chromosomal rearrangement within the...

Diagnostic Criteria According to the International Headache Society for Migraine Without Aura

Episodic attacks lasting 4 to 72 hours Two of the following symptoms 4. Moderate to severe intensity. AND one of the following symptoms during the headache 2. Photophobia and phonophobia. At least five attacks fulfilling the above criteria. Adapted with permission from Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders, 2nd ed. Cephalalgia 2004 24 S9-S60, and from Blackwell Publishing.

Modeling The Diagnostic Process And The Establishment Of Standardized Diagnostic Criteria

Diagnostic criteria are usually based on traditional teaching that may be influenced through time by the literature. The literature often reflects an informal distillation of diagnostic concepts held by clinical experts in the field. However, a few examples exist where an ad hoc declaration of diagnostic criteria for a condition have been widely accepted. For instance, the Jones criteria for the diagnosis of rheumatic fever, although revised intermittently (6), have been the accepted standard...

Neuromyelitis Optica

Also known as Devic's disease, neuromyelitis optica (NMO) has been considered a syndrome in its own right or a variant of MS. It has generally been considered a rare disease, but it is unclear if this is because of true rarity vs poor ascertainment methods, or whether its incidence varies by population. It has been estimated to cause about 5 of demyelinating disorders in Japan and India. All of the criteria listed in Table 6 agree that NMO must present with optic neuritis and myelitis, but the...

Acute panautonomic neuropathy

Sympathetic, parasympathetic nervous systems are involved. c. Cardiovascular involvement is common (postural hypotension, tachycardia, hypertension, dysrhythmias). d. Blurry vision, dry eyes, and anhydrosis. e. Recovery is gradual and often incomplete. f. Often combined with sensory features. Adapted from Newswanger DL, Warren CR. Guillain-Barre Syndrome. Am Fam Physician 2004 15 2405-2410. Available online at as a familial condition in up to 20 of cases. The prototypical disorder is adult...

Familial Hemiplegic Migraine

The clinical spectrum of Familial Hemiplegic Migraine associated with mutations in a neuronal calcium channel. N Engl J Med 2001 345 17-24. Ducros A, Joutel A, Vahedi K, Cecillon M, Ferreira A, Bernard E, et al. Mapping of a second locus for familial hemiplegic migraine to 1q21-q23 and evidence of further heterogeneity. Ann Neurol 1997 42 885-890. Joutel A, Bousser MG, Biousse V, Labauge P, Chabriat H, Nibbio A, et al. A gene for familial hemiplegic migraine...

With Subcortical Infarcts And Leukoencephalopathy

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is associated with mutations in the NOTCH 3 protein, which maps to chromosome 19q12. NOTCH signaling is important in development, but in adults, NOTCH 3 expression is limited to vascular smooth muscle cells, where its function is unknown. Pathologically, there are granular deposits in small cerebral arteries producing ischemic stroke because of vessel wall thickening, fibrosis, and occlusion....

Tension Headache

Lipton RB, Cady RK, Stewart WF, Wilks K, Hal C. Diagnostic lessons from the Spectrum Study. Neurology 2002 58 S27-S31. Ozge A, Bugdayci R, Sasmaz T, et al. The sensitivity and specificity of the case definition criteria in diagnosis of headache a school-based epidemiological study of 5562 children in Mersin. Cephalalgia 2003 23 138-145. Rossi LN, Cortinovis I, Menegazzo L, Brunelli G, Bossi A, Macchi M. Classification criteria and distinction between migraine and tension-type headache in...

Headache see specific sections in Chapter 8 for International Headache Society criteria

Migraine without aura Idiopathic, recurrent headache manifested by attacks lasting 4-72 hours. Typical characteristics are unilateral location, pulsating quality, moderate-to-severe intensity, aggravation by routine physical activity, and associated with nausea, vomiting, photo- and phonophobia. At least five attacks fulfilling the aforementioned criteria. ii. Migraine with aura Idiopathic, recurrent disorder manifested by attacks of neurological symptoms localizable to cerebral cortex or...

Aicardigoutieres Syndrome

Aicardi-Goutieres syndrome was first described in 1984 in a series of eight patients. The clinical picture is of infants with familial progressive encephalopathy, basal ganglia calcification, and chronic cerebrospinal fluid pleocytosis (Table 1). It is a very rare syndrome, suspected to be familial with autosomal-recessive inheritance, and occurrence in siblings has been reported. A majority of reported cases have elevated serum levels of a-interferon, which is of unclear pathogenic...

Cluster Headache

Cluster headache is a primary headache disorder of unclear etiology. It is more common in males, and is often precipitated by ingestion of alcohol. Not infrequently, the patient awakes from sleep with the onset of the headache. Patients are typically quite agitated during the attack, which tends to be relatively brief compared with the time-course of migraine or tension-type headaches. Cluster headaches may occur on an episodic or chronic basis. Chronic cluster headache may be primary or a...

Fraser Syndrome

Fraser syndrome is a rare constellation of signs of a multisystem congenital malformations described in Table 13. Slavotinek and Tifft have reviewed the known cases, and found that cryptophthalmos, syndactyly, and ambiguous genitalia are the most common signs. Friedreich's ataxia is the most common hereditary ataxia. It is a childhood-onset disorder, and one of its clinical hallmarks is absence of deep tendon reflexes. Direct genetic testing is now available, but the basic clinical criteria...

Headache Related To Intrathecal Injections

In the International Headache Society (IHS) classification category, headache related to intrathecal injections is a direct effect of the agent with the following diagnostic criteria (Table 6) if the headache is because of presumed chemical meningitis, the IHS criteria specify the criteria listed in Table 7. From a clinical standpoint, there may be diagnostic confusion of headache because of a low cerebrospinal fluid (CSF) pressure or post-lumbar puncture (LP) headache, as opposed to a pure...

Dementia With Lewy Bodies

Combining both features of a primary degenerative dementia and an akinetic-rigid, parkinsonian syndrome with prominent behavioral features, dementia with Lewy bodies (DLB) illustrates some of the Acute Intoxication Owing to Opioid Use A. The general criteria for acute intoxication must be met. B. There must be dysfunctional behavior, as evidenced by at least one of the following 6. Interference with personal functioning. C. At least one of the following signs must be present 3. Pupillary...

Parkinsons Disease

Albanese A, Diagnostic criteria for Parkinson's disease. Neurol Sci, 2003 24 S23-S26. Gelb DJ, Oliver E, Gilman S. Diagnostic criteria for Parkinson's disease. Arch Neurol 1999 56 33-39. Hedera P, Lerner AJ, Castellani R, Friedland RP. Concurrence of Alzheimer's disease, Parkinson's disease, diffuse Lewy body disease, and amyotrophic lateral sclerosis. J Neurol Sci 1995 128 219-224. Kompoliti K, Goetz CG, Boeve BF, et al. Clinical presentation and pharmacological therapy in corticobasal...

Corticobasal Degeneration

First described in the 1960s, corticobasal degeneration is also known as cortical-basal ganglionic degeneration. It typically comes on as an asymmetric parkinsonian syndrome, most often in the sixth decade or later. Long considered a rare disorder, the description of additional cases and case series in recent years has widened the clinical spectrum. Cortical manifestations of corticobasal degeneration include not only dementia and alien limb sign, but also apraxia and cortical sensory loss. The...

Exclusion Criteria for the Diagnosis of MSA

Symptomatic onset under 30 years of age. b. Family history of a similar disorder. c. Systemic diseases or other identifiable causes for features listed in Table 7. d. Hallucinations unrelated to medication. b. Prominent slowing of vertical saccades or vertical supranuclear gaze plasy.a c. Evidence of focal cortical dysfunction such as aphasia, alien limb syndrome, and parietal dysfunction. III. Laboratory investigation Metabolic, molecular, genetic, and imaging evidence of an alternative...

Diagnostic Categories of Multiple System Atrophy

Possible MSA one criterion plus two features from seperate domains. When the criterion is Parkinsonism, a poor levodopa response qualifies as one feature (hence only one additional future is required). II. Probable MSA criterion for autonomic failure urinary dysfunction, plus poor levodopa-responsive Parkinsonism or cerebellar dysfunction. III. Definite MSA pathologically confirmed by the presence of a high density of glial cytoplasmic inclusions in association with a combination of...

National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy Clinical

For possible and probable For possible and probable Gradually progressive disorder with age at onset at 40 or later Recent history of encephalitis alien limb syndrome cortical sensory deficits focal frontal or temporoparietal atrophy hallucinations or delusions unrelated to dopaminergic therapy cortical dementia of Alzheimer's type prominent, early cerebellar symptoms or unexplained dysautonomia or evidence of other diseases that could explain the clinical features Either vertical supranuclear...

Neuropsychiatric Systemic Lupus Erythematosus

In 1992, the ACR published a committee report on classification and case definitions of NPSLE. These case definitions were published in an appendix to the main article and are presented here in complete form. The committee recommended a basic demographic form for case-reporting purposes that is not included here. The syndromes can be subdivided into 19 syndromes encompassing both peripheral nervous system manifestations and CNS syndromes. Each of the guidelines is based on criteria with

Progressive Supranuclear Palsy

First described by Steele, Richardson, and Olzweski in 1964, progressive supranuclear palsy (PSP) is also known by its acronym. The original descriptions focused on the parkinsonian movement disorder with progressive eye movement abnormality and frequent falls. Dementia also occurs as part of the clinical expression of PSP. PSP is almost never familial in nature. More recent investigations have tended to widen the scope of the clinical spectrum, and clinical overlaps occur, particularly with...

Proposed Diagnostic Criteria for Parkinsons Disease

Clinical features according to diagnostic utility A. Group A Characteristic of Parkinson's disease B. Group B Suggestive of other diseases 1. Features unusual early in the disease a. Prominent postural instability within the first 3 years of the disease. b. Freezing phenomena in the first 3 years. c. Hallucinations unrelated to medications in the first 3 years. d. Dementia preceding motor symptoms or in the first year i. Supranuclear gaze palsy (other than upgaze restriction) or slowing of...

Fahrs Disease

Subacute dementia and imaging correlates in a case of Fahr's disease. J Neurol Neurosurg Psychiatry 2004 75 1163-1165. Lang AE. Corticobasal degeneration syndrome with basal ganglia calcification Fahr's disease as a corticobasal look-alike Mov Dis 2002 17 563-567. Lauterbach EC, Cummings JL, Duffy J, et al. Neuropsychiatric correlates and treatment of lenticulostriatal diseases a review of the literature and overview of research opportunities in Huntington's,...

Neurosweet Disease

Sweet's disease is an inflammatory dermatological condition characterized by malaise, fever, leukocytosis, and raised erythematous plaques. It is also known as acute febrile neutrophilic dermatosis. Painful, dull-red plaques are characteristic. Skin biopsies reveal deep dermal infiltration of mature neutrophils, spared epidermis, and absence of vasculitis. Sweet's disease may involve the nervous system in multiple ways, and these manifestations have been labeled Neuro-Sweet disease. Common...

Whipples Disease

Whipple's disease is caused by the bacterium Tropheryma whipplei. It most commonly affects the small intestine, where it produces a syndrome of chronic diarrhea and malabsorption. The central nervous system is sometimes also involved, and rarely is the only apparent site of disease. Within the nervous system, it may produce dementia and behavioral, visual changes, supranuclear gaze palsy, seizures, autonomic hypothalamic disturbances, oculomasticatory myorhythmia, and oculofacial-skeletal...

International Headache Society Diagnostic Criteria for Episodic and Chronic Cluster Headaches

Description Cluster headache attacks occurring in periods lasting 7 days to 1 year separated by pain-free periods lasting 1 month or longer. Diagnostic criteria A. Attacks fulfilling criteria A-E for Cluster headache. B. At least two cluster periods lasting 7-365 days and separated by pain-free remission periods of > 1 month. Comment The duration of the remission period has been increase in this second edition to a minimum of Description Cluster headache attacks occurring for more than 1 year...

Fatigue In Multiple Sclerosis

Fatigue in multiple sclerosis has been defined by the 1998 Paralyzed Veterans of America Multiple Sclerosis Council for Clinical Practice Guidelines as a subjective lack of physical and or mental energy that is perceived by the individual or caregivers to interfere with usual or desired activities. Acute fatigue is defined as new or significant increase in fatigue within the last 6 weeks and as a fatigue that limits functional activities or quality of life. From Current Clinical Neurology...

Alzheimers Disease

AD is the most common form of dementia, accounting for an estimated 65-75 of cases of dementia, especially in aged individuals. Dementia itself is a symptom, not a diagnosis. Dementia is defined as acquired loss of cognitive functioning, and occurs in clear consciousness. This distinguishes it from mental retardation developmental delay and cases where consciousness is fluctuating or impaired, such as delirium or coma. AD was also one of the first neurological disorders to have a set of...

Transient Global Amnesia

Transient global amnesia was first described by Fisher and Adams in 1964 as a transient event in which there is altered behavior with prominent memory loss. Patients with this condition are typically Diagnostic Criteria for Olfactory Reference Syndrome A. A preoccupation with imagined body odor (including halitosis) persisting despite reassurance. B. At some point during the course of the disorder, the person recognizes that the preoccupation (obsession compulsion) is excessive or unreasonable....

Diagnostic Criteria for Inadequate Sleep Hygiene

Complaint of insomnia or excessive sleepiness Presence of at least one of the following 1. Daytime naps at least twice a week. 2. Variable bedtime or awakening hours. 3. Frequent episodes (two to three times a week) of extended time in bed. 4. Routine use of products containing alcohol, tobacco, or caffeine in the period preceding bedtime. 5. Performance of exercise near bedtime. 6. Plans to become implicated in exciting or emotionally bothersome activities near bedtime. 7. Frequent use of the...

Diagnostic Criteria for Environmental Sleep Disorder

Complaint of insomnia or excessive sleepiness. 2. Complaint is temporally associated with the introduction of an environmental stimulus or circumstance that alters sleep and is physically measurable. 3. The physical properties of the environmental factor explain the sleep complaint the psychological significance of the environmental factor does not account for the complaint. 4. Withdrawal of the causal environmental factor leads to immediate or gradual resolution with a return to normal sleep....

Munchausen Syndrome By Proxy

Generally a pediatric diagnosis, Munchausen syndrome by proxy (MSBP) may be diagnosed both by inclusion and by exclusion. Table 57 identifies the specific criteria to be followed. Unfortunately, Neuropathological Subtypes in Frontotemporal Dementia 1. When the predominant neuropathological abnormalities are tau-positive inclusions (with associated neuron loss and gliosis), and insoluble tau has a predominance of tau with three microtubule-binding repeats, the most likely diagnoses are as...

Info

No criteria applied, retrospective clinical diagnosis. Validity values are given in percentages. DLB, dementia with Lewy bodies AD, Alzheimer's disease PD, Parkinson's disease PSP, progressive supranuclear palsy MSA, multiple system atrophy CBD, corticobasal degeneration VaD, vascular dementia Sens., sensitivity Spec., specificity PPV, positive predictive value NPV, negative predictive value K, kappa statistic (inter-rater reliability) Prob., probable Poss., possible H, hallucinations C,...

Fragile X Syndrome

Fragile X syndrome is the most common genetic cause for mental retardation and the most common known cause for autism. International Cowden Consortium Operational Criteria as of 2000 for the Diagnosis of Cowden's Syndrome d. Mucosal lesions. Major criteria 2. Thyroid carcinoma (nonmedullary), especially follicular thyroid carcinoma. 3. Macrocephaly (megalencephaly) (> 95th percentile). 4. Lhermitte-Duclos disease (LDD). 1. Other thyroid lesions (e.g., adenoma or multinodular goiter). 4....

Modified Dandy Criteria for Idiopathic Intracranial Hypertension

Signs and symptoms of increased intracranial pressure (headaches, nausea, vomiting, transient visual obscurations, papilledema). 2. No localizing neurologic signs, with the exception of unilateral or bilateral cranial nerve VI paresis. 3. Cerebrospinal fluid can show increased pressure, but otherwise without cytological or chemical abnormalities. 4. Normal to small symmetric ventricles must be demonstrated by neuroimaging. Note the typical patient profile (typical patient see Table 13) of a...

Diagnostic Criteria for Essential Tremor

Criteria for definite ET (all five must be true) 1. On examination, a + 2 postural tremor of at least one arm (a head tremor may also be present, but is not sufficient for the diagnosis). 2. On examination, there must be a. a + 2 kinetic tremor during at least four tasks, or b. a - 2 kinetic tremor on one task and a + 3 kinetic tremor on a second task tasks include pouring water, using a spoon to drink water, drinking water, touching finger to nose, and drawing a spiral. 3. If on examination...

Current Clinical Neurology

Diagnostic Criteria in Neurology, edited by Alan J. Lerner-, 2006 Sleep Disorders in Women From Menarche Through Pregnancy to Menopause, edited by Hrayr P. Attarian, 2006 Psychiatry for Neurologists, edited by Dilip V. Jeste and Joseph H. Friedman, 2006 Status Epilepticus A Clinical Perspective, edited by Frank W. Drislane, 2005 Thrombolytic Therapy for Acute Stroke, Second Edition, edited by Patrick D. Lyden, 2005 Parkinson's Disease and Nonmotor Dysfunction, edited by Ronald F. Pfeiffer and...