Paraplegia

Paraplegia is sensory or motor disorder in the lower extremities of the body. The impairment is due to damage caused as a result of congenital deformities or trauma and spinal injuries. The effects of paraplegia are predominantly seen in the thoracic, lumbar and sacral region. The damage caused as a result of paraplegia can lead to other issues such as monoplegia (dysfunction of one organ), paralysis and some times fecal incontinence and impotence.

Spastic paraplegia and flaccid paraplegia are two important forms of the disease which are differentiated based on the location. Flaccid paraplegia is associated with the lesion caused in the spinal cord; which in turn causes two significant forms of myelopathies compressive and non-compressive respectively. The compressive form of paraplegia occurs because of the destruction of the spinal cord due to pressure exerted on it. In most cases the pressures caused are because of underlying medical conditions such as neoplasms, degenerative spinal disease or hematoma. The compressive forms are predominantly located in extradural and subdural regions causing extra medullary and intramedullary complications such as neurofibroma and meningioma.

The lesions caused affect the spinal arteries causing ischemia eventually leading to tissue necrosis in the respective region. This leads to the development of edema in the spinal region causing compression and damage. Because of increased pressure, paraplegia can initiate complications such as pneumonia, pressure sores which are decubitus in origin. Often the pain associated with paraplegia radiates in the region of nerve damage. Vitamin B 12 deficiency also leads to the occurrence of paraplegia of non compressive origin.

Meningioangiomatosis

This is a rare malformation of the central nervous system, so rare that not many cases of cystic Meningioangiomatosis (MA) have been reported. First described by Bassoe and Nuzum in 1915, Meningioangiomatosis was found incidentally in an autopsy of a 15-year old boy. It is characterized by a plaque-like or nodular mass overlapping leptomeninges in patients.

Though the pathogens are unknown, there are three possible theories – first, these lesions probably represent a hamartoma - a benign malformation made up of an abnormal mixture of cells and tissues. Second, they could result from direct invasion of the brain tissue by a leptomeningeal meningioma. Third, they represent a vascular malformation.

A rare disease characterized by the brain, benign lesion of the leptomeninges, MA usually involves the cerebral cortex. Seizures are often noticed in these patients. Though extremely rare multifocal cases have been reported both in adults and children, the lesion is normally found in one place. Biopsy is necessary for diagnosis and conventional treatment is to surgically remove the lesion.

Meningioangiomatosis may either be sporadic or associated with Neurofibromatosis type 2. In sporadic Meningioangiomatosis, seizures and headaches are present. Whereas, NF2 is asymptomatic and diagnosed only at autopsy. Meningioangiomatosis is benign and the seizures can be surgically corrected.

Analysis

When medical records of ten histopathologically confirmed MA patients were treated between 2010 and 2011, all the patients presented with symptomatic seizure attacks before surgery. MRI and CT were the radiological examinations done for pre-operative diagnosis of all cases. Accurate diagnosis of Meningioangiomatosis is important because it is a benign surgically correctable cause of seizures.

Results

All patients underwent craniotomy surgeries with total gross resections of MA lesions.

Post operative follow-ups range from 8 to 108 months. No radiological recurrence was found in any case. While eight patients achieved total symptomatic remission after surgeries, two were found to suffer from attacks infrequently under severe anti-epileptic drugs.

Although MA is rare and usually misdiagnosed, it is possible for correct preoperative diagnosis with differential diagnosis taking into consideration patient's ages, symptomatic seizure attacks, and radiological findings. MA is curable and the prognosis is excellent since patients become free of seizure after surgical treatments.