|
Senior Member (jccglutenfree)
|
|
Join Date: Aug 2006
Location: Wisconsin
Posts: 1,581
|
|
|
Senior Member (jccglutenfree)
Join Date: Aug 2006
Location: Wisconsin
Posts: 1,581
|
Some excerpts:
Quote:
Examination revealed right-sided spastic hemiparesis with a pyramidal pattern of leg weakness (Medical Research Council Grade 4-/5 in hip flexion, 4+/5 in hip extension, 4/5 in knee flexion and 4-/5 in ankle dorsiflexion) associated with mild wasting of the right quadriceps. The patient had generalized bilateral hyperreflexia, sustained right ankle clonus and a right extensor plantar response. Results of cranial nerve, cerebellar and sensory examinations were normal.
T2 and fluid-attenuated inversion recovery brain MRI sequences revealed a region of hyperintensity along the course of the left corticospinal tract, arising from the subcortical white matter of the precentral gyrus and following the posterior limb of the internal capsule into the brainstem (Figure 1A). Gadolinium-enhanced MRI did not reveal any contrast enhancement. Repeat neuroimaging 2 months later revealed more-extensive changes in the same pattern, with additional involvement of the opposite (right) subcortical region of the precentral gyrus (Figure 1B).
Coronal fluid-attenuated inversion recovery MRI of the patient's brain demonstrating regions of hyperintensity at initial presentation and 2 months later, with partial resolution following 9 months on a gluten-free diet. (A) Initially the hyperintensity is confined to the subcortical white matter of the left motor region, descending via the internal capsule into the brainstem. (B) Two months later there is more-extensive hyperintensity visible in the left hemisphere, and limited involvement of the right motor cortex. (C) After 9 months on a gluten-free diet, the hyperintensity of the left corticospinal tract is more confined, and the right motor cortical changes have resolved. Neuroradiological improvement was accompanied by substantial clinical improvement of the patient's right-sided hemiparesis.
Electromyography (EMG) of the masseter, biceps, first dorsal interosseous extensor digitorum communis and the vastus medialis muscles demonstrated widespread fasciculations, reduced recruitment of motor units, and frequent complex polyphasic waveforms. Fibrillation potentials were recorded in the right vastus lateralis. Results of nerve conduction studies were normal. Cerebrospinal fluid analysis revealed that all constituents were normal and a polymerase chain reaction test for JC virus was negative.
Routine blood tests revealed a mild microcytic anemia (hemoglobin 129 g/l, normal range 120-140 g/l; mean corpuscular volume 78 fl, normal range 80-98 fl) with low levels of serum iron (3.5 µmol/l [20 g/dl], normal range 45-300 µmol/l [251-1,676 g/dl]), serum ferritin (17 pmol/l; normal range 45-675 pmol/l) and serum folate (8.6 nmol/l, normal range 7-40 nmol/l). Results of a subsequent antiendomysial antibody test were positive (IgA level 5.88 g/l). Duodenal biopsy analysis demonstrated villous atrophy, crypt hyperplasia and increased intraepithelial lymphocytes (Marsh 3A), consistent with gluten-sensitive enteropathy (celiac disease). Tests for HIV type 1 and 2 antibodies were negative.
The patient was started on a gluten-free diet approximately 7 months after the onset of his initial neurological symptoms. No drugs, including riluzole or other agents with neuroprotective potential, were given. At a follow-up examination 9 months after initiation of treatment, the patient's right arm function, assessed by the neurologist who performed the initial examination, had returned to normal. Improvement in the patient's right leg function was more limited, wasting was still present and there was some residual spasticity. He was now able to walk unaided, however, and his handwriting and ability to fasten buttons had returned to normal. Repeat MRI demonstrated partial resolution of the corticospinal tract changes (Figure 1C). EMG examination was not repeated after treatment. The patient's antigliadin antibody response fell from pretreatment levels of IgA 34 U/ml and IgG 44 U/ml (normal <15 U/ml for both) to IgA 18 U/ml and no detectable IgG antibody. The patient was actively followed up for 2.5 years after his initial presentation, with no evidence of neurological relapse.
In this Case Study, the patient's initial presentation consisted of a progressive motor syndrome in the absence of sensory signs, with clinical evidence of upper and lower motor neuron degeneration, electromyographic evidence of widespread acute denervation, and hyperintensity in the corticospinal tracts revealed by MRI. In view of these findings, while acknowledging the unusual hemiparetic presentation and strikingly territorial nature of the white matter changes seen on MRI, a diagnosis of amyotrophic lateral sclerosis (ALS) was initially considered by the referring neurologist. The apparent presentation of a rare ALS variant in association with celiac disease -- a condition with various neurological manifestations -- made it entirely appropriate, however, to review the initial diagnosis and institute treatment for celiac disease. Ultimately, improvement in the patient's symptoms following treatment for celiac disease rendered the diagnosis of ALS untenable.
|
Quote:
As there is currently no cure for ALS and the mean survival time from symptom onset is 3-4 years, it is important to consider all potentially treatable diseases that might produce a similar syndrome. Clinicians should recognize, however, that in practice such 'mimics', although understandably appealing to anxious patients and their carers, are extremely rare.
|
__________________
.
|
10-06-2007, 08:36 PM
Reply With Quote
Similar Threads
Linear Mode
