Unusual neurological diseases

______ is seen in 30-50% of patients with corticobasal degeneration

alien limb phenomenon

may affect arm or leg

the limb moves outside of voluntary control and is able to perform complex action

the limb has “a mind of its own”

alien limb phenomenon

visual anosognosia (denial of vision loss) associated with confabulation to mask the deficit

Etiology is damage to bilateral occipital lobes producing cortical blindness

the pt lacks awareness of their deficit

very rare condition

anton-babinksi syndrome (or just anton syndrome)

Pt presents with neuropsychiatric symptoms

an autoimmune encephalitis that may be associated with ovarian teratoma or other rare tumor

may present with unusual movement disorders

NMDAR encephalitis

often presents with gait abnormality due to lower extremity dystonia (may be mistaken for cerebral palsy or spastic diplegia)

has diurnal fluctuation: symptoms may worsen in afternoon and improve in the morning after sleep

remarkable response to low dose levodopa

dopa responsive dystonia or Segawa disease or DYT 5

you see dystonic posture or dyskinesia that is brief (seconds to minutes)

attacks triggered by sudden involuntary movements, stress, startle, or sleep deprivation

seizures may also be present

responds to carbamazepine

(this was the one where half of the man’s body seized up when he stood up)

paroxysmal kinesigenic dyskinesia

result of a patient who has had a lesion of the corpus callosum

this was the case where the man could not identify the familiar object in his left hand but could in his right hand

split brain syndrome or callosal disconnection syndrome

acute onset of anterograde amnesia (inability to form new memories)

repeatedly asks the same question reflecting disorientation

other cognitive functions are normal and they eventually return to normal

presumed to be either migraine, ischemic, epileptic, or psychogenic in origin

transient global amnesia

occurs when an individual falsifies physical or psychological signs or symptoms or induces injury or diseases with intended deception

may lie, put drops of blood in urine, or inject themselves with bacteria

behavior persistent in the absence of monetary rewards or drugs

factitious disorder

most severe variant of factitious physical disorder

Munchausen’s syndrome

form of child or elder abuse

the caregiver causes the sickness

Munchausen by proxy

characterized by a preoccupation with a perceived flaw or defect in the patient’s appearance

body dysmorphic disorder

false belief that a patient is pregnant including a growing abdomen, morning sickness, and cessation of menstruation

unclear if due to hormones or in some cases may be psychiatric

pseudocyesis

neurologic symptoms such as weakness, abnormal movements, or nonepileptic seizures without structural disease

disorder causes distress and/or functional impairment and has a poor prognosis

functional neurological disorder

the patient consciously creates physical or psychological symptoms to assume the sick role with the goal of perceiving reward (not the goal to achieve “patient hood”)

not a psychiatric illness

malingering

excessive concern about having or developing a serious undiagnosed general medical disease

previously known as hypochondriasis

illness anxiety disorder

seizures involve abnormal electrical impulses within the cerebral cortex

this is the case where the woman was kissing the cross around her neck and unresponsive for several minutes

may consist of automatisms (lip smacking, chewing, etc.) or stereotypies

focal seizure with dyscognitive features

characterized by sudden and time-limited disturbances of motor, sensory, autonomic, cognitive, and or emotional functions that can mimic epileptic seizures

this was the case about the lady who was asked about her troublesome son and begins to shake violently

these events are not caused by abnormal brain activity (are caused by physical or mental stress)

non-epileptic seizures (dissociative)

inability to recognize faces

runs in families and does not involve a brain lesion

congenital prosopagnosia

a perceptual disorder involving brief transient episodes of visual distortions

can occur in conjunction with viral infections (EBV most common)

objects or body arts are perceived as altered in various ways

no permanent brain abnormalities are seen

Alice in Wonderland Syndrome

Painful sensations referred to the missing limb (could also be breast or rectum)

females and upper extremity amputation are at greater risk

mirror therapy is used

phantom limb syndrome