Search

A delayed syndrome of progressive weakness has been described in survivors of paralytic poliomyelitis - "Post-Polio Muscular Atrophy (PPMA)". One proposed etiology is a drop-out of motor neurons due to increased metabolic demands of an enlarged motor unit territory. We report a patient with slowly progressive lower extremity weakness 20 years after recovery from an episode of myelopathy involving the lower lumbar and sacral segments of the spinal cord. Delayed progressive amyotrophy may complicate any significant injury to anterior horn cells.

We describe a patient who developed, over a 22-month period, a giant aneurysm of his basilar artery. A prior MRI of the brain done for nonspecific symptoms showed a normal brainstem and basilar artery. At presentation, he had a repeat MRI scan for a 4-month history of a partial right oculomotor nerve palsy and left hemiparesis. The MRI revealed a giant aneurysm of the top of the basilar artery. This was treated by angiographic placement of Guglielmi detachable coils (GDC) after surgical intervention was deemed unfeasible. This case illustrates the acquired nature of intracranial aneurysms. All inoperable intracranial aneurysms should be closely monitored and MRI and MR angiography may currently be the best noninvasive methods for this purpose. Intra-arterial GDC embolization of aneurysms is an alternative treatment when surgery is not possible.

A 56-year-old female presented with mild low back pain. Examination revealed severe, selective atrophy of the thoracic and lumbar paraspinal muscles. Fibrillations were seen in the paraspinal muscles on EMG. Limb EMG was normal. Biopsy of the gluteus maximus was normal. Paraspinal muscle biopsy revealed neurogenic features. Atrophy of the thoracic and lumbar paraspinal muscles was noted on magnetic resonance imaging. This patient has selective amyotrophy of the thoracic and lumbar paraspinal muscles. This may be an addition to the spectrum of 'benign focal amyotrophy'. The differential diagnosis of paraspinal muscle weakness is discussed.

We report a biopsy-proven case of sarcoidosis limited to the spinal cord and responsive to treatment with oral corticosteroids. Involvement of the spinal cord by systemic sarcoidosis is uncommon and is associated with several unusual pathological and radiological characteristics that may contribute to its misdiagnosis and subsequent mismanagement. The importance of these unusual characteristics is greatly amplified in solitary spinal cord sarcoidosis because there are no systemic findings to suggest a sarcoid etiology.

Although receptors for somatostatin are found in bone cells, the effect of somatostatin analogs on calcium metabolism is unknown. The authors studied, in a metabolic ward, the effect of octreotide (a long-acting somatostatin analog) and a placebo in two 6-day calcium balance periods in 8 children with Duchenne muscular dystrophy. As expected, octreotide (2 micrograms/kg, subcutaneously, every 8 hours) reduced serum growth hormone and somatomedin (IGF-1) to levels found in growth hormone deficiency. Octreotide enhanced calcium retention by 30% (96 mg daily [P < 0.04]) in 7 boys for whom complete data (diet, urine, and fecal calcium) were available. In 6 children with urinary calcium excretion (Uca) greater than 50 mg daily, octreotide markedly lowered Uca, from 114 +/- 23 mg daily to 61 +/- 9 mg daily (P < 0.03). Calcium retention occurred in patients with or without initial hypercalciuria, but the higher the basal Uca, the greater was the inhibition by octreotide (r = 0.79; P < 0.03). Inactive, nonambulatory patients had a more pronounced response of Uca to octreotide (P < 0.02). Octreotide caused a mild, nonsignificant reduction in fecal calcium, with no major changes in serum calcium, phosphorus, parathyroid hormone, urinary excretion of sodium and potassium, or in creatinine clearance. Based on the current observations and the presence of receptors for somatostatin in bone cells, this hormone may have, at least on a short-term basis, an anabolic effect on calcium, perhaps favoring its deposition in bone.

Myositis ossificans developed in a 67-year-old man recovering from tetanus. This rare complication develops in the convalescent phase of tetanus through metaplastic changes in undifferentiated connective tissue cells in areas of myocellular injury. Anoxia, hematoma formation, and immobilization may be contributing factors. Permanent loss of joint motion may be the end result of this ossifying process.