Neuro case

Candmed debat
#1

A 40 year-old right-handed man complained of dysfunction in his right arm of about 6 month’s duration. Onset was gradual and associated with slurring of speech, shaking of his left hand during use, and coughing and sputtering when swallowing liquids. His father and brother had similar symptoms: after 10 years, his father became confined to a wheelchair. The patient has never consumed alcohol.The general physical exam and mental status were normal. Speech was dysarthric. Coarse nystagmus was evoked by conjugate gaze in any direction. Smooth pursuit was abnormal and saccades were dysmetric. Sensory exam was normal. Tone was mildly diminished in all limbs. No resting tremor was noted, but there was a side-to-side intention tremor of both hands, right greater than left. There was minimal difficulty with the heel-to-shin maneuver. Rhythmic hand motions were decomposed (i.e. the patient exhibited dysdiadochokinesis). Muscle strength and muscle stretch reflexes were normal. Babinski’s sign was not present. Gait was somewhat broadbased and unsteady.

Localize the lesion or lesions and What is the likely diagnosis in this case?

Det var en svær en, for mig i det mindste.

hvad synes i?

#2

Hmm - og det skulle være almen viden at kunne fyre en diagnose af på den case?

Du har måske svaret?

#3

prøv nu at give et bud og evt. elaborate hvorfor du har valgt diagnosen. det er ikke så svært som det umildbart ser ud til fra starten, hvis man tænker lidt over det hinter alle symptomerne mod et struktur i hjernen.

#4

Jeg synes det er lidt forvirrende…

intentionstremor og dysdiadochokinesi peger i retning af noget cerebellart, men samtidig er der tale- og synsforstyrrelser :roll:

Måske en rumopfyldende proces, som trykker på radiatio optica og påvirker cerebellum gennem tentorium? Dunno… :oops:

Hvad er svaret? :shock:

#5

Hmm. Er ening, i at det må være i lillehjernen. Intention tremor, dysdiadochokinese, dysartri og hypotoni tyder på lillehjernehemisfærelæsion. Den bredsporede gang kunne tyde på vermis dysfunktion.
Men der behøver vel strengt taget ikke være hæmisfærepåvirkning!

Måske er det en af disse arvelige cerebellare sygedomme.

Bare et bud. Den er lidt svær!

#6

obstruktion af a. basiliaris og manglende blodforsyning medfølgende iskæmi i de forsynende områder: cerebellum og baghjernen…er mit bud. :slight_smile:

#7

Good shots guys

answer–

lesions---->The intention tremor, dysdiadochokinesis, dysmetria, dysarthria and hypotonia all suggest cerebellar hemispheric involvement and the broad-based gait is indicative of some midline (cerebellar vermis) dysfunction.The physical findings suggest a diffuse or multifocal involvement of the cerebellar hemispheres with some involvement of midline cerebellar structures as well.

Diagnosis—>Given the strongly positive family history, indolent onset pattern and predominant pattern of cerebellar hemispheric dysfunction, the most likely diagnosis is a familial cerebellar or spinocerebellar degenerative process

Diseases associted with cerebellar syptoms could be–

Rostral vermis syndrome due to chronic alcoholism (not in this case though)

Intoxications

Posterior circulation vascular disease

Multiple sclerosis (not in this case)

Neoplasm

Paraneoplastic autoimmune syndromes

Infections or autoimmune post-infectious syndromes

Developmental abnormalities of the cerebellum

Metabolic derangements (such as aminoaciduria or hypothyroidism)

#8

Dysdiadokokinesia (difficulty in performing rapid alternating movements)
ataxia (difficulty in coordinating movements)
tremors
loss of balance and vertigo
muscle weakness
dysarthria (loss of coordination of the muscles controlling speech)
loss of postural tone.

Tyder på læsion i cerebellum

#9

Followup spørgsmålet er hvorfor er der ikke lesion i basal ganglia?

#10

On a side note:
Dem af jer der godt kan lide en case i ny og næ:
http://www.emedicine.com
Jeg får tilsendt en case engang imellem…