Pick Disease Clinical Presentation

Updated: Jun 06, 2014
  • Author: A M Barrett, MD; Chief Editor: Jasvinder Chawla, MD, MBA  more...
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Presentation

History

The onset of behavioral and cognitive dysfunction in individuals with Pick disease can be difficult to identify.

The primary impairment in cognition normally does not involve an abnormal level of consciousness or distractibility. Such a finding is more consistent with an attentional dementia [15] or a confusional state and/or dementia. In many cases, individuals with Pick disease perform well on quick cognitive screening tests, such as the Mini-Mental Status Examination (MMSE). [11] However, these studies omit assessment of behavioral and social judgment or response inhibition, and, thus, this kind of quick screening is not sensitive to early Pick disease impairment that can still be disabling.

Clinical course during the first 2 years

Behavioral variant

Psychiatric abnormalities that seem to respect the pattern of the classic frontal lobe syndromes are present. [16] Patients with orbitofrontal dysfunction can become aggressive and socially inappropriate. They may steal or demonstrate obsessive or repetitive stereotyped behaviors. Patients with dorsomedial or dorsolateral frontal dysfunction may demonstrate a lack of concern, apathy, or decreased spontaneity. Patients can demonstrate an absence of self-monitoring, abnormal self-awareness, and an inability to appreciate meaning. [16] Patients with gray matter loss in the bilateral posterolateral orbitofrontal cortex and right anterior insula may demonstrate changes in eating behaviors, such as a pathologic sweet tooth. Patients with more focal gray matter loss in the anterolateral orbitofrontal cortex may develop hyperphagia. [17]

Primary progressive aphasia

Speech and language abnormalities often begin early and progress rapidly. Memory impairment is relatively less severe than speech/language and behavioral changes.

Across subtypes of FTD, including Pick disease, patients can demonstrate apathy, irritability, and agitation. [18] Patients may be depressed early in the disease. These mood changes can predate amnesia.

Patients usually have relatively little limb apraxia and/or visuospatial dysfunction, thus distinguishing them from patients with diffuse, bihemispheric impairment.

Incontinence can occur early. In contrast, continence generally is preserved in mild-to-moderate Alzheimer disease.

Parkinsonism, with its concomitant history of rigidity and gait impairment, can occur. Severe parkinsonism suggests an alternate diagnosis, such as corticobasal ganglionic degeneration, diffuse Lewy body disease, or progressive supranuclear palsy.

Next:

Physical Examination

The general physical examination often shows the patient to be unkempt at an earlier stage than in comparably impaired patients with Alzheimer disease.

Abnormal spontaneous behaviors observed during examination may include the following:

  • Witzelsucht or inappropriate jocularity
  • Echolalia (repeating the examiner's words), echopraxia (imitating the examiner's gestures), [19, 20] and other disinhibited approach or utilization behaviors

A general neurologic examination may include some of the following abnormalities:

  • Primitive reflexes, such as grasp, suck, and snout (not the palmomental reflex, which is often present in healthy individuals [18] )
  • Akinesia, plastic rigidity, or paratonia on motor examination [21]
  • Resting tremor - Uncommon; its presence suggests Parkinson disease or a Parkinson-plus syndrome

Mental status/neuropsychological examination may reveal the following:

  • Verbal output that is often nonfluent - Most patients have difficulty in naming common objects or pictures (anomia); spontaneous speech can be sparse, yet fluent, in character, with preserved grammar (logopenia)
  • Perseveration - Cognitive and motor (see the image below)
    Motor perseveration in a patient with Pick disease Motor perseveration in a patient with Pick disease. The patient was asked to copy loops (as demonstrated by the examiner in the first line).
  • Relatively preserved visuospatial and visual orientation skills
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