Symptomatic Chiari type 1 malformation associated with acromegaly: A case report

Background: Here, we report a patient who presented with both symptomatic acromegaly and symptomatic Chiari I malformation (CM1) with a C2-T5 syrinx. Case Description: A 63-year-old female presented with bilateral arm dysesthesias and back pain. For approximately the past 30 years, she had chronic signs of acromegaly (i.e. an enlarged forehead, jaw, and nose, and enlarged hands and feet). When the cervical magnetic resonance showed a CM1 (tonsillar herniation) with C2-T5 syringomyelia, she underwent foramen magnum decompression and C1 posterior arch resection. Postoperatively, she was asymptomatic. The added finding of a growth hormone (GH)-producing pituitary lesion was treated medically with endocrine therapy, as she had incidentally required surgery/chemotherapy for a newly diagnosed colon cancer. Conclusion: Symptomatic CM1, syrinx, and acromegaly may occur together. Appropriately treatment may include a suboccipital decompression, and C1 arch resection surgery, followed by either surgical or medical treatment for the GH-producing pituitary adenoma.

Downbeat Nystagmus Secondary to Chiari I Malformation

Context: Downbeat nystagmus are characterized by the rapid phase in the down direction in primary position of the eyes, in the most cases represents a cerebellar dysfunction, typically with injuries involving vestibulocerebellum (flocculus, paraflocculus, nodule and uvula), although most cases are due to primary lesions in the brain stem, usually involving paramedian tracts. This type of nystagmus is characteristic of craniocervical abnormalities, such as Chiari 1 malformation, also being seen in a wide variety of cerebellar diseases, including degenerative etiologies, toxic-metabolic and ischemic injury. Case report: A 45-year-old woman, who presented with dizziness started 6 years ago, related to rapid head movement, with progressive worsening, leading to difficulty in walking and a tendency to fall to the right, associated with mild to moderate intensity occipitonuchal headache, with improvement at rest and horizontal decubitus. Neurological examination showed downbeat nystagmus, intent tremor in the finger-to-nose-test, worse on the right arm, Romberg test presentes with anteroposterior and lateral instability, in addition to Tanden with bilateral lateropulsion, without other relevant changes. The magnetic resonance imaging showed platybasia and vertebrobasilar invagination, Chiari type 1 malformation with tonsillar herniation, without signs of cervical hydrosyringomyelia, signs of atrophy of both cerebellar hemispheres, a nonspecific nodule in the subcortical region of the left precuncle. Conclusion: The case described above is relevant to demonstrate that even in cases of acute changes in adulthood, screening for anatomical malformations in the posterior fossa should be considered.

Acquired Chiari Type 1 Malformation Secondary to Paget’s Disease of the Bone: A Case Report

Chiari type 1 malformations are characterised by caudal descent of cerebellar tonsil into foramen magnum. Usually it is congenital. Paget’s disease causing acquired chiari type 1 malformation is rare with only five reported cases in the literature. The diagnosis is primarily by Magnetic Resonance Imaging (MRI). Authors reported a case of acquired chiari type 1 malformation due to paget’s disease in a 58-year-old male patient diagnosed by MRI and Computed Tomography (CT). The patient presented with complaints of headache, unsteady gait, slow movements with weakness in all the four limbs, difficulty in walking which aggravated since three days and stammering of speech. Initially a diagnosis of motor neuron disease was suspected and the patient was subjected for MRI examination of brain and spine. MRI revealed platybasia with cerebellar tonsillar herniation with calvarial thickening and multiple vertebral collapse. The CT of brain and spine was done to evaluate bones specifically in view of thickened cranial vault and multiple vertebral collapse on MRI which revealed features of paget’s disease in the form of widened sclerotic bone with lytic areas and thickened trabeculae. Hence, diagnosis of paget’s disease causing acquired chiari type 1 malformation was made which correlated with biochemical findings. The management is different in primary and acquired chiari type 1 malformation and hence it is important to diagnose this condition which is relatively rare.