Pick Disease

 Pick Disease

overview 

When we think about dementia, we usually picture memory loss as the first sign. Pick's disease, and FTDs altogether, remind us that dementia has other faces as well. Language, personality, and behavior, affected early in Pick's disease and other frontal lobe dementias can deteriorate before prominent memory changes are noted. For clinicians and caregivers, this is a reminder that cognition is a broader term than memory, and that changes in personality or language, not just memory changes, require careful evaluation.

Causes

People with FTD have abnormal substances (called tangles, Pick bodies, Pick cells, and tau proteins) inside nerve cells in the damaged areas of the brain.

The exact cause of the abnormal substances is unknown. Many different abnormal genes have been found that can cause FTD. Some cases of FTD are passed down through families.

FTD is rare. It can occur in people as young as 20. But it usually begins between ages 40 and 60. The average age at which it begins is 54.

Symptoms

The disease gets worse slowly. Tissues in parts of the brain shrink over time. Symptoms such as behavior changes, speech difficulty, and problems thinking occur slowly and get worse.

Early personality changes can help doctors tell FTD apart from Alzheimer disease. (Memory loss is often the main, and earliest, symptom of Alzheimer disease.)

People with FTD tend to behave the wrong way in different social settings. The changes in behavior continue to get worse and are often one of the most disturbing symptoms of the disease. Some persons have more difficulty with decision-making, complex tasks, or language (trouble finding or understanding words or writing). There are variations of FTD seen with other nervous system problems such as:

*Amyotrophic Lateral Sclerosis (ALS/Lou Gehrig disease)

*Primary progressive aphasia

*Progressive supranuclear palsy

General symptoms include:

BEHAVIORAL CHANGES:

*Not able to keep a job

*Compulsive behaviors

*Impulsive or inappropriate behavior

*Inability to function or interact in social or personal situations

*Problems with personal hygiene

*Repetitive behavior

*Withdrawal from social interaction

*EMOTIONAL CHANGES

*Abrupt mood changes

*Decreased interest in daily living activities

*Failure to recognize changes in behavior

*Failure to show emotional warmth, concern, empathy, sympathy

*Inappropriate mood

*Not caring about events or environment

*LANGUAGE CHANGES

*Cannot speak (mutism)

*Decreased ability to read or write

*Difficulty finding a word

*Difficulty speaking or understanding speech (aphasia)

*Repeating anything spoken to them (echolalia)

*Shrinking vocabulary

*Weak, uncoordinated speech sounds

NERVOUS SYSTEM PROBLEMS

*Increased muscle tone (rigidity)

*Memory loss that gets worse

*Movement/coordination difficulties (apraxia)

*Weakness

OTHER PROBLEMS

*Urinary incontinence

*Exams and Tests

*The health care provider will ask about the medical history and symptoms.

Tests may be ordered to help rule out other causes of dementia, including dementia due to metabolic causes. FTD is diagnosed based on symptoms and results of tests, including:

*Assessment of the mind and behavior (neuropsychological assessment)

*Brain MRI

*Electroencephalogram (EEG)

*Examination of the brain and nervous system (neurological exam)

*Examination of the fluid around the central nervous system (cerebrospinal fluid) after a lumbar puncture

*Head CT scan

*Tests of sensation, thinking and reasoning (cognitive function), and motor function

*Newer methods that test brain metabolism or protein deposits may better allow for more accurate diagnosis in the future

*Positron emission tomography (PET) scan of brain

*A brain biopsy is the only test that can confirm the diagnosis, although usually never recommended.

Diagnosis

There are abundant symptom overlaps between frontotemporal dementia and other neuropsychological disorders. Psychiatric conditions such as depression, bipolar disorder, and, most significantly, schizophrenia can mimic behavioral variant frontotemporal dementia and are often misdiagnosed during early presentations of frontotemporal disease. Onset and progression patterns can assist in differentiating schizophrenia from frontotemporal dementia. While frontotemporal dementia generally occurs in patients over forty-five years of age, initial presentations of schizophrenia are observed in younger age groups. A consistently advancing disease course characterizes frontotemporal dementia, while schizophrenia progression is variable and can plateau.

Distinguishing other dementia syndromes from frontotemporal dementia can also be challenging. A predominance of memory and visuospatial deficits suggests the more prevalent Alzheimer's dementia variant. Lewy body dementia should be considered in the setting of visual hallucinations and parkinsonism. 

Several miscellaneous conditions can present with neuropsychiatric and behavioral symptoms similar to frontotemporal dementia including, infections, toxins, neoplasms, trauma, paraneoplastic disease, autoimmune disorders, thyroid syndromes, hepatic encephalopathy, and various genetic pathologies.

Treatment:

Treatment using medications developed for AD sometimes aggravates the symptoms of FTDs. There is no specific medication for FTDs. Medications for FTD, therefore, are “off label” and symptom-oriented rather than disease-modifying or curative.

For challenging behaviors, non-pharmacologic therapy remains the most recommended approach. Antidepressant medications such as citalopram or sertraline are sometimes tried empirically. They have helped some patients but exacerbated the symptoms of others. Aricept® (donepezil) or Exelon® (rivastigmine), often the first medication for AD patients, have been reported to have inconsistent effects on people with FTDs. Sometimes they help, but sometimes they aggravate the symptoms. Similarly, the NMDA antagonist, Namenda® (memantine), has been reported helpful to some FTD patients and adverse to others.

Antipsychotic medications have sometimes been prescribed in an attempt to control impulsive or agitated behavior, but there is no consistent evidence to support their use in this way. Antipsychotic use should be clinically justified and combined with clear expectations regarding these medications’ possible risks and desired benefits.

TYPE OF DOCTOR AND DEPARTMENT:  Hepatologist  SPECIALIST CAN DIAGNOSES THIS DISEASE.