History and Diagnosis

In our series, about one-third of patients experienced some form of pain as their first symptom. The remainder complained about sensory deficits, paresthe-sias, motor weakness, or gait problems as their first manifestation in about equal proportions. With malignant tumors, almost all patient complained about pain, gait ataxia, or motor weakness right form the start (Table 3.2). In general, the case history averaged about 33±44 months, with considerable variability, ranging between 1 week and 28 years. Benign tumors had a significantly longer history compared to malignant tumors (36±46 months and 7±13 months, respectively; p<0.0001).

Approximately 36% of tumors were located in the cervical cord, 50% in the thoracic cord, and the remaining 14% in the conus area [98]. According to our experience, the advent of MRI has not led to a faster diagnoses in general (30±32 months before and 33±41 months after the introduction of MRI). After all, intramedullary tumors are so rare that they are quite often not included in the differential diagnosis when the first symptoms have appeared. However, patients are in better neurological condition at the time of surgery now compared to the pre-MRI era [356]. In our series, the average preoperative Kar-nofsky score has improved from 55±20 to 70±17 (p=0.0001) since the introduction of MRI. Patients who for years are thought to have multiple sclerosis while neurological symptoms continue to progress due to an overlooked intramedullary tumor, should be a phenomenon of the past.

The average age at presentation was 38±17 years. The typical clinical history was characterized by a gradual and slow progression without intermittent remission of symptoms. This is a very important point for the differential diagnosis to inflammatory or de-myelinating syndromes. The latter almost always develop significant deficits quickly, within days or weeks, and then fluctuate and display some variability in terms of intensity and/or localization of symptoms with time. Sudden clinical progressions occur in intramedullary tumors only in exceptional cases. We have encountered them in association with either tumor hemorrhages [4, 163, 242, 301, 324] or highly malignant tumors [130].

On admission, the clinical situation had progressed to affect motor functions to some degree for at least 80% of patients. Motor weakness or a gait problem was considered their major concern for 22% and 38% of patients, respectively. Even though pain was still present in 52% of patients, just 19% wanted surgery predominantly for pain relief. Sensory deficits were found in 87% of cases and dysesthesias in 56% (Table 3.3). The preoperative Karnofsky score averaged about 67±18. In the literature, most studies describe a progressive course of neurological deterioration prior to surgery. However, unusual presentations due to affections of the autonomic nervous system are also reported. Fricke and Romine [102] described a patient with a thoracic ependymoma presenting with orthoTable 3.2. Initial symptoms of intramedullary tumors

First symptom

Benign tumors

Malignant Adults tumors









Gait ataxia






Motor weakness






Sensory deficits


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