Ataxia

Question 1

Symptoms of ataxia

Answer 1

Poor coordination

Unsteady gait and stumbling

Difficulty with fine motor tasks

Changes in speech

Involuntary nystagmus

Difficulty swallowing

Question 2

Symptoms of cerebellar ataxia

Answer 2

Dysdiadochokinesis (inability to perform movements that involve rapidly altering hand position)

Ataxia (clumsiness, unsteady wide based gait, difficulty with fine motor control)

Nystagmus (un voluntary rapid eye movements)

Intention tremor (task specific)

Slurred speech (dysarthia)

Heel-shin test positive (poor coordination)

Question 3

GF diet to treat gluten ataxia

Answer 3

Shows neurophysiological improvement of symptoms regardless of presence of enteropathy.

GF diet works as long as you are definitely gluten sensitive

GI symptoms may occur, weight may go down, unsteady feeling, symptoms can relapse

If 1 blood test shows positive antibodies then repeat after 6 months, if a patient is run down then the antibodies will be positive but if they are feeling better they may not show up so always repeat the test

Improvement of ataxia associated with adherence to gluten-free diet

Suggests that gluten ataxia is another manifestation of, and not merely an association with gluten sensitivity. Improved ataxia in patients without enteropathy suggests that other organ specific manifestations can be triggered/perpetuated by the ingestion of gluten without enteropathy

Question 4

Diagnosis of gluten ataxia

Answer 4

Presence of serological markers of sensitivity to gluten (AGA, TG6, TG2, EMA)

Gluten ataxia + AGA is 18% of all ataxias

Gluten ataxia is 47% of idiopathic sporadic ataxias

Question 5

Monitoring gluten ataxia

Answer 5

MR spectroscopy to monitor patients (looks at cerebellar structure and function) - NAA measure tells you about metabolic activity of the area of interest, the higher the better. This varies patient to patient so need creatine (doesn’t change in disease). NAA: creatine ration - NAA should be higher. Abnormal results show NAA well below creatine. Suggests reduced neuronal density and cell death in cerebellum.

Diet improves ratio in all patients with reduced antibodies but if patients don’t follow a strict diet and still have + antibodies then their ratio will get worse. Improvement of ataxia within 1 year on GF strict diet even those without enteropathy.

Question 6

Gluten neuropathy

Answer 6

26% of all neuropathies

2 types: sensorimotor axonal symmetrical and sensory ganglionopathy (33%)

symptoms: decreased/loss of feeling, difficulty using arms/hands/legs/feet, tingling, numbness, burning

GF diet - neurophysiological evidence of improved neuropathy within 1 year of adherence to strict GF diet

Question 7

Gluten encephalopathy

Answer 7

Episodes of severe intractable headaches

White matter abnormalities on MRI

GF diet - improves headache but not WM changes

No GF diet - can progress to vascular dementia

General defect in the way the brain is thinking and working = encephalopathy

Question 8

Coeliac disease

Answer 8

Gluten sensitivity that is an enteropathy

Diagnosed by intestinal biopsy

Anti-endomysial and anti-gliadin antibodies +

Question 9

Enteropathy and gluten sensitivity

Answer 9

Dermatitis Herpetiforms (a manifestation of gluten sensitivity) can occur without histological evidence of an enteropathy