Diagnosis of Alexander Syndrome

Alexander Syndrome Diagnosis: Book Excerpts

• Ask the following questions - DEMENTIA
• Differential Diagnosis - Dementia
• Approach to the Diagnosis - MEMORY LOSS AND DEMENTIA
• History and physical examination - Muscle spasticity [Muscle hypertonicity]
• History and physical examination - Gait, spastic [Hemiplegic gait]
• History and physical examination - Muscle spasticity [Muscle hypertonicity]
• History and physical examination - Gait, spastic [Hemiplegic gait]
• History. - Dementia
• Differential Overview - Dementia
• History - Muscle spasticity
• History and physical examination - Muscle spasticity [Muscle hypertonicity]
• History and physical examination - Gait, spastic [Hemiplegic gait]
• Approach to the Diagnosis - MEMORY LOSS AND DEMENTIA
• Approach to the Diagnosis - SPASTICITY

Diagnostic Tests for Alexander Syndrome: Online Medical Books

16 MEDICAL BOOKS ONLINE! Review excerpts from medical books online, free, without registration, for more information about diagnostis of Alexander Syndrome.

DEMENTIA: Ask the following questions:
(Algorithmic Diagnosis of Symptoms and Signs)

1. Is there a history of drug or alcohol ingestion? Chronic barbiturate intoxication, ergotism, and other psychotropic or antidepressant drugs may cause dementia. Alcoholism may cause dementia in the form of Korsakoff's psychosis or Wernicke's encephalopathy.
2. Is there headache, papilledema, or focal neurologic signs? The most important condition to rule out in this category would be a space-occupying lesion, but normal pressure hydrocephalus, cerebral arteriosclerosis, acute cerebrovascular accident, and general paresis may present with focal neurologic signs.
3. Is there a response to niacin, thiamine, vitamin B ₁₂ , or thyroid? Response to these drugs would indicate that the patient has pellagra, Korsakoff's psychosis, pernicious anemia, or myxedema. However, laboratory tests should be done before administering the medications. Laboratory tests include serum B ₁₂ and folic acid, and a thyroid profile. Unfortunately, most laboratories do not have a test for niacin or thiamine.
4. Is there insight? In patients with cerebral arteriosclerosis, the patient notices that his memory is slipping. This is also true of acquired immunodeficiency syndrome (AIDS).
5. Are there extrapyramidal tract signs? Extrapyramidal tract signs should suggest Huntington's chorea or Parkinson's disease.
6. Are there pyramidal tract signs or myoclonus? Pyramidal tract signs are seen in general paresis and Jakob-Creutzfeldt syndrome, but also myoclonus is seen in Jakob-Creutzfeldt syndrome.

DIAGNOSTIC WORKUP

Routine laboratory tests include a CBC, sedimentation rate, chemistry panel, VDRL test, HIV antibody titer, ANA, blood alcohol level, urine drug screens, thyroid profile, serum B ₁₂ , and folic acid. A CT scan should probably be done in all cases. An EEG may be helpful in demonstrating drug intoxication. A spinal tap may need to be done to diagnose central nervous system lues. The best test for that is the fluorescent treponema antibody absorption test (FTA-ABS). MRI may be useful in distinguishing Alzheimer's disease from cerebral arteriosclerosis, as in cerebral arteriosclerosis small infarcts may be demonstrated. A radioiodinated serum albumin (RISA) cisternography study is useful to diagnose normal pressure hydrocephalus. Arterial blood gases should be drawn. Psychiatric testing will help differentiate organic brain syndrome from other psychiatric disorders and malingering. A neurologist or psychiatrist should be consulted before ordering expensive diagnostic tests.

» READ BOOK EXCERPT ONLINE »

Source: Algorithmic Diagnosis of Symptoms and Signs, 2003

Dementia: Differential Diagnosis
(In a Page: Signs and Symptoms)

• Alzheimer's disease is the most common cause of dementia
• Lewy body dementia
• Multi-infarct dementia
• Parkinson's disease
• Alcohol/drugs
• Vitamin deficiency (B₁₂, thiamine)
• CNS infections
  –HIV encephalitis
  –Meningitis
  –Herpes encephalitis
  –Creutzfeldt-Jacob disease
  –Cerebral abscess
  –Neurosyphilis
• Depression (pseudodementia)
• Head trauma
• Pick's disease
• Chronic subdural hematoma
• Huntington's disease
• Chronic hydrocephalus
• Paraneoplastic encephalitis
• Hypothyroidism
• Cerebral vasculitis
• Systemic lupus erythematosus (lupus cerebritis)
• Wilson's disease
• Chronic hypoglycemia or hypocalcemia
• Uremic encephalopathy
• Dialysis dementia
• Multiple sclerosis
• Hydrocephalus
• Postanoxic dementia

Workup and Diagnosis

• Important to distinguish dementia from delirium (acute metabolically induced state of fluctuating consciousness) and depression
• A complete history and physical are essential to rule out underlying medical, neurologic, or psychiatric illnesses that may mimic symptoms of dementia
  –Mini-mental status exam
  –Medication history should be elicited to identify drugs that may contribute to cognitive changes (e.g., analgesics, sedatives, anticholinergics, antihypertensives)
  • Labs may include CBC, electrolytes, calcium, BUN/creatinine, liver function tests, glucose, thyroid function tests, vitamin B₁₂ and folate, screening for inflammatory/infectious causes, and toxicology screen
  • CT without contrast to rule out structural lesions (e.g., infarct, malignancy, hydrocephalus, extracerebral fluid collection)
  • EEG is not routinely used; however, it may identify toxic/metabolic disorders or Creutzfeldt-Jakob disease
  • Genetic testing may be indicated if family history suggests Alzheimer's disease (especially early-onset)
    –Mutations of chromosomes 1, 14, 21
    –Increased frequency of apolipoprotein ∊ 4 allele
    • CSF analysis may be useful in some cases
    • HIV and syphilis (RPR) testing if known risk factors

» READ BOOK EXCERPT ONLINE »

Source: In a Page: Signs and Symptoms, 2004

MEMORY LOSS AND DEMENTIA: Approach to the Diagnosis
(Differential Diagnosis in Primary Care)

Once again, the presence or absence of other neurologic signs and symptoms is important. If one does not have the skills or the time for a complete neurologic examination, immediate referral is indicated. Next, a careful drug history is done. Withdrawal of all drugs may clear the dementia. An EEG, skull x-ray film, CT scan or MRI, spinal tap (if there is no papilledema), and psychometric tests are basic to any workup. If the CT scan or MRI shows dilated ventricles, a spinal fluid nuclear flow study is indicated to exclude normal-pressure hydrocephalus. In the absence of other neurologic signs and spinal fluid analysis negative for syphilis and other chronic encephalopathies, one should do an endocrine workup and look for systemic diseases such as porphyria. Drug screens for lead intoxication, and bromism should also be performed.

» READ BOOK EXCERPT ONLINE »

Source: Differential Diagnosis in Primary Care, 2007

Muscle spasticity [Muscle hypertonicity]: History and physical examination
(Handbook of Signs & Symptoms (Third Edition))

When you detect spasticity, ask the patient about its onset, duration, and progression. What, if any, events precipitate its onset? Has he experienced other muscular changes or related symptoms? Does his medical history reveal an incidence of trauma or a degenerative or vascular disease?

Take the patient’s vital signs, and perform a complete neurologic examination. Test reflexes and evaluate motor and sensory function in all limbs. Evaluate muscles for wasting and contractures.

During your examination, keep in mind that generalized spasticity and trismus in a patient with a recent skin puncture or laceration indicates tetanus. If you suspect this rare disorder, look for signs of respiratory distress. Provide ventilatory support, if necessary, and monitor the patient closely.

» READ BOOK EXCERPT ONLINE »

Source: Handbook of Signs & Symptoms (Third Edition), 2006

Gait, spastic [Hemiplegic gait]: History and physical examination
(Handbook of Signs & Symptoms (Third Edition))

Find out when the patient first noticed the gait impairment and whether it developed suddenly or gradually. Ask him if it waxes and wanes, or if it has worsened progressively. Does fatigue, hot weather, or warm baths or showers worsen the gait? Such exacerbation typically occurs in multiple sclerosis. Focus your medical history questions on neurologic disorders, recent head trauma, and degenerative diseases.

During the physical examination, test and compare strength, range of motion (ROM), and sensory function in all limbs. Also, observe and palpate for muscle flaccidity or atrophy.

» READ BOOK EXCERPT ONLINE »

Source: Handbook of Signs & Symptoms (Third Edition), 2006

Muscle spasticity [Muscle hypertonicity]: History and physical examination
(Professional Guide to Signs & Symptoms (Fifth Edition))

Once you detect spasticity, ask the patient about its onset, duration, and progression. What, if any, events precipitate onset? Has he experienced other muscular changes or related symptoms? Does his medical history reveal any incidence of trauma or degenerative or vascular disease?

Take the patient’s vital signs, and perform a complete neurologic examination. Test reflexes and evaluate motor and sensory function in all limbs. Evaluate muscles for wasting and contractures.

During your examination, keep in mind that generalized spasticity and trismus in a patient with a recent skin puncture or laceration indicates tetanus. If you suspect this rare disorder, look for signs of respiratory distress. Provide ventilatory support, if necessary, and monitor the patient closely.

» READ BOOK EXCERPT ONLINE »

Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006

Gait, spastic [Hemiplegic gait]: History and physical examination
(Professional Guide to Signs & Symptoms (Fifth Edition))

Find out when the patient first noticed the gait impairment and whether it developed suddenly or gradually. Ask him if it waxes and wanes or if it has worsened progressively. Does fatigue, hot weather, or warm baths or showers worsen the gait? Such exacerbation typically occurs in multiple sclerosis. Focus your medical history questions on neurologic disorders, recent head trauma, and degenerative diseases.

During the physical examination, test and compare strength, range of motion, and sensory function in all limbs. Also, observe and palpate for muscle flaccidity or atrophy.

» READ BOOK EXCERPT ONLINE »

Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006

Dementia: History.
(The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter)

A concerned family member will often bring patients into the office when obvious memory impairment occurs. The deficit will be sufficiently obvious to interfere with daily living, work, or social activities. When questioning the reliable historian, ask about the specific cognitive changes (language, judgment, abstract thinking, praxis, visual recognition, and constructional ability). Ask about the duration of symptoms, the mode of onset (insidious or abrupt), and the progression (slow or rapid, gradual or stepwise). Check on general risk factors (e.g., increasing age, atherosclerosis, head trauma, CNS infection), and family history. Specific risk factors for AD include increasing age, lower intelligence, small head size, history of head trauma (4), and Down’s syndrome.

Physical examination.

The patient should undergo a thorough general and neurologic examination and Mini-Mental Status Examination (MMSE). Look for focal neurologic deficits and assess cognitive function (memory, language, perception, praxis, attention, judgment, calculation and visuospatial function). Other neuropsychiatric testing is available.

» READ BOOK EXCERPT ONLINE »

Source: The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter, 2000

Dementia: Differential Overview
(Field Guide to Bedside Diagnosis)

❑ Alzheimer disease

❑ Multi-infarct dementia

❑ Depression

❑ Drugs

❑ Parkinson disease

❑ Frontal lobe dementia

❑ Vitamin B₁₂ deficiency

❑ HIV encephalopathy

❑ Korsakoff syndrome

❑ Brain tumor

❑ Normal pressure hydrocephalus

❑ Chronic subdural hematoma

❑ Neurosyphilis

❑ Creutzfeldt-Jakob

❑ Wilson disease

Diagnostic Approach

Many patients are concerned about age-related forgetting of details, a normal phenomenon. This usually results from decreased attention. The fact that they recognize and worry about this distinguishes them from patients with early dementia. Normal forgetting preserves vocabulary and spelling and improves with cues. For example, patients with Alzheimer disease cannot recall a list of related words any better than random words. Patients with dementia on the other hand have difficulty with one or more of the following: learning and retaining new information (e.g., remembering events); handling complex tasks (e.g., balancing a checkbook); reasoning (e.g., inability to cope with unexpected events); spatial orientation (e.g., getting lost in familiar places); language (e.g., word finding); and/or behavior.

Subtle impairments in memory, attention, and concentration are often easily compensated for and therefore hard to pinpoint. Impaired judgment and abstraction on increasingly simple matters and personality changes (notably irritability) are usually noted first. The time course of onset is helpful in distinguishing dementia from delirium, but acute exacerbations of an underlying dementia that mimic delirium are common with drugs and acute physical illness.

The Mini Mental State Exam (MMSE) measures across domains of cognitive function: memory, executive function, attention, language, praxis, and visuospatial ability. A total score of less than 24/30 suggests dementia or delirium. Scores of 20 to 24 suggest mild impairment; 16 to 19, moderate; and 15 or below, severe. It also provides a quantitative assessment useful in following the course of the disease or response to therapy.

Mini-Cog Repeat three objects, Clock Drawing Test (hands at 8:20), then recall three objects. Suggestive of dementia when either 0/3 objects are recalled or when 1 to 2 are missed and CDT is abnormal.

» READ BOOK EXCERPT ONLINE »

Source: Field Guide to Bedside Diagnosis, 2007

Muscle spasticity: History
(Signs & Symptoms: A 2-in-1 Reference for Nurses)

If you detect spasticity, ask the patient about its onset, duration, and progression. What, if any, events precipitate onset? Has he experienced other muscular changes or related symptoms? Does his medical history reveal any incidence of trauma or degenerative or vascular disease?

» READ BOOK EXCERPT ONLINE »

Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007

Muscle spasticity [Muscle hypertonicity]: History and physical examination
(Nursing: Interpreting Signs and Symptoms)

When you detect muscle spasticity, ask the patient about its onset, duration, and progression. What, if any, events precipitate its onset? Has he experienced other muscular changes or related symptoms? Does his medical history reveal an incidence of trauma or a degenerative or vascular disease?

Take the patient's vital signs, and perform a complete neurologic and musculoskeletal examination. Test reflexes and evaluate motor and sensory function in all limbs. Evaluate muscles for wasting and contractures.

Alert

During your examination, keep in mind that generalized spasticity and trismus in a patient with a recent skin puncture or laceration indicates tetanus. If you suspect this rare disorder, look for signs of respiratory distress. Provide ventilatory support, if necessary, and monitor the patient closely.

» READ BOOK EXCERPT ONLINE »

Source: Nursing: Interpreting Signs and Symptoms, 2007

Gait, spastic [Hemiplegic gait]: History and physical examination
(Nursing: Interpreting Signs and Symptoms)

Find out when the patient first noticed the gait impairment and whether it developed suddenly or gradually. Ask him if it waxes and wanes, or if it has worsened progressively. Does fatigue, hot weather, or warm baths or showers worsen the gait? Such exacerbation typically occurs in multiple sclerosis. Focus your medical history questions on neurologic disorders, recent head trauma, and degenerative diseases.

During the physical examination, test and compare strength, range of motion (ROM), and sensory function in all limbs. Also, observe and palpate for muscle flaccidity or atrophy.

» READ BOOK EXCERPT ONLINE »

Source: Nursing: Interpreting Signs and Symptoms, 2007

MEMORY LOSS AND DEMENTIA: Approach to the Diagnosis
(Differential Diagnosis in Primary Care)

Once again, the presence or absence of other neurologic signs and symptoms is important. If one does not have the skills or the time for a complete neurologic examination, immediate referral is indicated. Next, a careful drug history is done. Withdrawal of all drugs may clear the dementia. An electroencephalogram (EEG), skull x-ray film, computed tomography (CT) scan or magnetic resonance imaging (MRI), spinal tap (if there is no papilledema), and psychometric tests are basic to any workup. If the CT scan or MRI shows dilated ventricles, a spinal fluid nuclear flow study is indicated to exclude normal-pressure hydrocephalus. In the absence of other neurologic signs and negative spinal fluid analysis for syphilis and other chronic encephalopathies, one should do an endocrine workup and look for systemic diseases such as porphyria. Drug screens for lead intoxication and bromism should also be performed.

» READ BOOK EXCERPT ONLINE »

Source: Differential Diagnosis in Primary Care, 2007

SPASTICITY: Approach to the Diagnosis
(Differential Diagnosis in Primary Care)

After the level of the lesion is established, an MRI or CT scan of that area can be ordered. A neurologist should be consulted first. A spinal tap will be useful in establishing the diagnosis of multiple sclerosis, encephalitis, and neurosyphilis if a space-occupying lesion has been ruled out.

» READ BOOK EXCERPT ONLINE »

Source: Differential Diagnosis in Primary Care, 2007

 » Next page: Signs of Alexander Syndrome

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