A Question For The Mds

[Jestgar]

Would any of you considered testing this woman for Celiac?

(I believe this requires subscription to nejm to fully open)

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[Jestgar]

I decided to post just the symptoms. I don't think that would be a copyright infringement.

A 58-year-old right-handed woman with type 1 diabetes was admitted to the hospital because of a 2-week history of increasing fatigue and word-finding difficulties and a 2-day history of right-arm weakness.

She had been in her usual state of health until 3 years before admission, when an episode of word-finding difficulty occurred, associated with headache and mild right-sided facial weakness. She was admitted to a local hospital, where computed tomography (CT) of the head revealed no abnormalities. Magnetic resonance imaging (MRI) revealed a punctate subcortical lesion in the left parietal white matter; magnetic resonance angiography (MRA) revealed no extracranial or intracranial stenosis. The symptoms resolved spontaneously within a few hours. Anticoagulation therapy with heparin followed by warfarin was begun, and she was discharged on the third hospital day. A 24-hour Holter monitor showed no arrhythmia. Several similar episodes occurred over the next 5 months, accompanied by weakness in the right arm and leg, which again resolved in a few hours. Repeated MRI and CT of the head, electrocardiography, and 48-hour cardiac monitoring showed no new abnormalities.

Two years before admission, the patient saw a neurologist at this hospital because of continuing episodes of confusion, word-finding difficulty, right-sided weakness, and fatigue, often accompanied by headaches with photophobia, nausea, and vomiting. Right-sided headaches had occurred intermittently for the past 5 years, preceded by flashes of light in the periphery of her visual fields. Since the first episode of word-finding difficulty, the headaches had been occasionally accompanied by tingling in the right fingers and forearm. She reported clumsiness of her right hand and difficulty with attention, calculation, and memory. Neurologic examination at that time showed diminished peripheral vision bilaterally, mild right ptosis, diminished sensation to pinprick and to light touch on the right side of the face, and a flattened right nasolabial fold. Motor strength was 4/5 in the hands and feet bilaterally and 5/5 elsewhere; deep-tendon reflexes were 1/4 distally and 2/4 proximally. There was no Babinski's sign. Warfarin was discontinued, and aspirin therapy was initiated, after which the patient was free of symptoms through a 1-month follow-up.

Eighteen months before admission, another episode of forgetfulness, word-finding difficulty, and right-sided weakness occurred. MRI showed a 1-cm2 area of increased signal on T2-weighted images of the periventricular white matter of the left occipital lobe, as well as nonspecific changes in periventricular white matter. One year before admission, the patient was admitted to her local hospital because of another strokelike episode. MRI showed areas of hyperintensity on T2-weighted images of the left corona radiata and of the splenium of the corpus callosum, which did not enhance after the administration of contrast material. On lumbar puncture, the cerebrospinal fluid was found to have normal serum chemistry and cell counts; a test for cryptococcal antigen was negative, and one oligoclonal band was detected. Hypercoagulability testing was negative. Aspirin and extended-release dipyridamole were begun.

Five months before admission, the woman was again seen by a neurologist at this hospital. Findings on neurologic examination were unchanged. Repeated MRI showed on T2-weighted images patchy areas of hyperintensity involving the periventricular white matter and the pons, with more prominent lesions in the left corona radiata and corpus callosum. Routine serum chemistry testing, tests for antinuclear antibodies and Lyme antibody, and vitamin B12 levels were normal.

Three months before admission, headache and word-finding difficulties recurred, and the patient was admitted to this hospital. Repeated MRI showed a new area of hyperintensity on T2-weighted images in the posterior left corona radiata. Positron-emission tomography with 18F-fluorodeoxyglucose showed diffuse hypometabolism in the cerebral cortex. Magnetic resonance spectroscopy showed no abnormalities. Lumbar puncture was performed to collect cerebrospinal-fluid specimens, for which serum chemistry, cell counts, cultures, and electrophoresis were normal. Tests for anti-Ro and anti-La antibodies, for IgG antibodies against hepatitis C, for hypercoagulability, and for methylmalonic acid were negative, as were serum and urine toxicology screens and genetic tests for the NOTCH3 mutation and for mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). Skin and muscle biopsies were performed; pathological examination showed thickening of the basal lamina around small blood vessels, but a specific diagnosis was not made. Weekly injections of interferon beta-1a were begun. The patient was discharged to a rehabilitation facility on the 10th hospital day and went home 3 weeks later.

Two months later, she again had fatigue. During a 2-week period, she thought that her right-sided weakness had progressed, and on the day of admission, she had another episode of word-finding difficulty, headache, and weakness in both hands. She came to the emergency department of this hospital. While there, she became agitated, mute, and unable to follow commands. In response to noxious stimuli, she grimaced and withdrew her right arm but not her right leg. The results of CT and CT angiography were normal. She was admitted to the hospital.

Diabetes had been diagnosed 30 years earlier, during pregnancy. Glycemic control had been difficult to achieve. The patient had been treated at various times with multiple daily insulin injections and insulin-pump therapy, and on admission she was receiving a basal bolus regimen of insulin glargine, 18 units at night, and insulin lispro before meals according to a sliding scale. Her median glycated hemoglobin level over the previous 4 years was 9.1% and had ranged from a low of 6.8% to a high of 11.2%. Selected laboratory-test values are shown in Table 1. Systemic complications of diabetes included autonomic neuropathy with orthostatic hypotension and severe gastroparesis, peripheral neuropathy, nephropathy, and retinopathy. She had hypertension, coronary artery disease with a history of a silent myocardial infarction, congestive heart failure, and intermittent atrial fibrillation. A diagnosis of hypothyroidism had been made 35 years earlier. Other medical problems included depression, hepatitis B, shoulder and hip bursitis, and a duodenal ulcer.

[Canadian Karen]

I saw this in my NEJM newsletter I get and even with just the 100 word abstract, it screamed out celiac to me!

Karen

[tarnalberry]

I don't think it screams celiac at all. I'm not saying that it couldn't be, but it really isn't all that similar to the pattern that is common (not even most common) for celiac. And this is a reason it's hard to diagnose - the pattern is very disperse. There are a couple of things that, once other options are exhausted, say "yeah, test for that too", but no, not top of the list. And *definitely* not a first presentation.

But I'm no doctor.

[Guest Doll]

Yes. I would screen her for Celiac. But that doesn't mean that's her main or only problem. Long-term Type 1 diabetes damages the brain and nerves, and she also has advanced heart disease. She sounds overall in rough shape, and her known problems could account for some her symptoms. Her A1c was *horrible*. She might also be developing MS or some form of autoimmune vasculitis. Could be Celiac, but could also be more than that or something else.

[Jestgar]

I found it curious that it wasn't even part of the differential...