Mexican standoff
What’s making this grumpy man’s jaw hurt?
Vol.18, No.07, August 2010

Franco was a 78-year-old Italian man with a lifelong fondness for the grape who also happened to have diabetes. He was just about controlled with metformin, running blood sugars around 7 to 10.

Last year, while in Mexico, he had an episode of syncope and was hospitalized for 2 days. He’d become disoriented, had difficulty swallowing and speaking for 48 hrs, and showed some transitory right-handed weakness. In Mexico, he was prescribed an additional blood pressure pill. He was also started on clopidogrel for possible TIA, stroke, query hypertensive encephalopathy, as his BP had clocked in at 210/110!

On his return to Canada, the usual stroke protocol investigations had been done, including CT brain scan, which only revealed cerebellar atrophy and changes consistent with his age, but no localized lesions. Carotid dopplers were normal; his neurological exam was basically normal also, except for confusion, which was now more noticeable, according to his wife (long-suffering lady that she was, and continues to be). His BP was normal.

Just to further complicate the picture, he’d been prescribed trifluoperazine (Stelazine) by a previous doctor, that seemed to help him as a soporific, as well as smoothing out his sometimes volatile moods. He’d been on trifluoperazine for 25 years.

Everything seemed to be unfolding as it should until I referred him to the neurologist, who unexpectedly diagnosed him with early parkinsonism, and started him on Sinemet 100/25 (carbidopa-levodopa), after noting that he didn’t swing his arms when walking, and showed some very mild cogwheeling. The neurologist also noted the probability of a syncopal/stroke episode, and continued the clopidogrel and the BP treatment begun in Mexico.

The neurologist also referred him to an expert in movement disorders for confirmation and ongoing treatment. This second specialist commented on developing tardive dyskinesia with repetitive tongue movements. He recommended tapering and stopping the trifluoperazine, as it also might contribute to the parkinsonism. This was done, but then he couldn’t sleep, so trazodone was started. Results were poor, however. The drug was too strong, according to his wife.

Now began the real challenge. Franco began complaining of increasing pain in his right ear and jaw. An ear and throat exam proved negative. Franco declined a jaw MRI to look for temporomandibular joint disorder.

The following week he looked a tad jaundiced, and complained of fatigue and increasing jaw pain, though the involuntary tongue movements had decreased. I was stumped, so he was sent to emergency where another negative CT scan was reported. It was at this moment that the casualty officer made a brilliant diagnosis, finally laying his finger on Franco’s problem. Can you guess what it was?

ANSWER
Trigeminal neuralgia

Franco was diagnosed with trigeminal neuralgia (TN) (tic douloureux, prosopalgia). This condition is diagnosed in about 1 in 15,000 people, usually after age 50, but probably many cases are missed. It’s frequently misdiagnosed as toothache, and some patients have a whole row of teeth removed before getting the right diagnosis! It’s common in multiple sclerosis, but most TN sufferers don’t have MS. It can be provoked by post-herpetic neuralgia. The commonest primary cause is nowadays believed to be trigeminal nerve compression by an enlarged blood vessel inside the skull. Carbamazepine is a standard first-line treatment for TN — but watch for skin reactions in Asians and especially Han Chinese.

In this case, it was initially treated with gabapentin 300 mg, daily in divided doses to start, and when I saw Franco in the office the next week, he said his pain was a little better — though he was still driving his wife crazy with irritability. The following week he again saw the movement specialist, who concurred with the diagnosis of trigeminal neuralgia but suggested gradually increasing the gabapentin to 600 or even 900 mg, as necessary. This brought very heartwarming results for all, not least Franco’s wife!

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