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Relapsing Remitting Multiple Sclerosis in an Iranian Patient with Neurofibromatosis Type I.

Mohebi N, Moghaddasi M, Maryam Z - Neurol Int (2015)

Bottom Line: These two disease do not share any apparent pathological similarities.We herein present a 32-year-old woman with definite NF-1, who has recently been diagnosed with MS, which to the best of our knowledge is a rare co-occurrence.Though there are often neurologic sign and symptoms in patients with NF-1, they should not always be considered as the natural history of the disease, and other overlapped pathologies should be kept in mind, in order to not miss or postpone the efficient treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Iran University of Medical Science , Tehran, Iran.

ABSTRACT
Neurofibromatosis type 1 (NF-1) is a common hereditary neuro-cutaneous disease, with known gene mutations, that mainly involves the skin and nervous system. Multiple sclerosis (MS) is an acquired inflammatory disease in which the myelin of nerve cells in the brain and spinal cord is damaged. These two disease do not share any apparent pathological similarities. We herein present a 32-year-old woman with definite NF-1, who has recently been diagnosed with MS, which to the best of our knowledge is a rare co-occurrence. Though there are often neurologic sign and symptoms in patients with NF-1, they should not always be considered as the natural history of the disease, and other overlapped pathologies should be kept in mind, in order to not miss or postpone the efficient treatment.

No MeSH data available.


Related in: MedlinePlus

T2/W cervical magnetic resonance imaging showing a hypersignal intramedullary demyelinating plaque (A, red arrow), with enhancement on T1 with gadolinium (B).
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fig002: T2/W cervical magnetic resonance imaging showing a hypersignal intramedullary demyelinating plaque (A, red arrow), with enhancement on T1 with gadolinium (B).

Mentions: A 32-year-old Iranian woman, known case of NF-1, was admitted to Rasoul-Akram Hospital, affiliated to Iran University of Medical Science, with weakness of right arm and distal paresthesia of both hands since the previous week. She did not have lower limbs weakness nor visual or sphincter problem. Her past history was remarkable for a self-limited binocular diplopia with no eye pain, congestion or visual loss and a history of vertigo accompanied with imbalance, five months and two weeks prior to her admission respectively. The past drug history was negative. Her family history was remarkable for her mother and three sisters suffering from NF-1. The physical examination of skin revealed diffuse cafe-au-lait spots in variable sizes, axillary freckling and multiple subcutaneous neurofibromas (Figure 1). Her mental state was normal. The motor force of right arm was 4/5 with normal sensation and generalized hyperreflexia was detected. Other neurologic examination including cranial nerves, fundoscopic exam and motor and sensory systems of other limbs were entirely normal. Magnetic resonance imaging (MRI) of the cervical spinal cord, with and without gadolinium (GD), was performed showing an intraaxial hypersignal lesion with enhancement (Figure 2). In addition, brain MRI, with and without GD revealed multiple hypersignal lesions in T2-weighted and fluid attenuated inversion recovery (FLAIR) sequences in periventricular and juxtacortical white matter and cerebellum with enhancement (Figure 3), which all were compatible with demyelinating plaques in MS and obviously distinct from non-enhancing unidentified bright objects occasionally seen symmetrically in the basal ganglia, brain stem and cerebellum in NF-1.3 Brain MRI also showed an asymptomatic large extra-axial avidly enhancing lesion at left side of the neck probably an intracranial neurofibroma or shwannoma (Figure 4); however, no biopsy was performed. Infectious etiologies such as varicella zoster virus, herpes simplex virus and cytomegalovirus were tested by polymerase chain reaction (PCR) in cerebrospinal fluid (CSF) and all were negative. Oligoclonal bands (OCB) was positive in CSF and visual evoked potential was normal bilaterally. The patient symptoms improved significantly with methylprednisolone pulse therapy with total dose of 5 grams.


Relapsing Remitting Multiple Sclerosis in an Iranian Patient with Neurofibromatosis Type I.

Mohebi N, Moghaddasi M, Maryam Z - Neurol Int (2015)

T2/W cervical magnetic resonance imaging showing a hypersignal intramedullary demyelinating plaque (A, red arrow), with enhancement on T1 with gadolinium (B).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4591495&req=5

fig002: T2/W cervical magnetic resonance imaging showing a hypersignal intramedullary demyelinating plaque (A, red arrow), with enhancement on T1 with gadolinium (B).
Mentions: A 32-year-old Iranian woman, known case of NF-1, was admitted to Rasoul-Akram Hospital, affiliated to Iran University of Medical Science, with weakness of right arm and distal paresthesia of both hands since the previous week. She did not have lower limbs weakness nor visual or sphincter problem. Her past history was remarkable for a self-limited binocular diplopia with no eye pain, congestion or visual loss and a history of vertigo accompanied with imbalance, five months and two weeks prior to her admission respectively. The past drug history was negative. Her family history was remarkable for her mother and three sisters suffering from NF-1. The physical examination of skin revealed diffuse cafe-au-lait spots in variable sizes, axillary freckling and multiple subcutaneous neurofibromas (Figure 1). Her mental state was normal. The motor force of right arm was 4/5 with normal sensation and generalized hyperreflexia was detected. Other neurologic examination including cranial nerves, fundoscopic exam and motor and sensory systems of other limbs were entirely normal. Magnetic resonance imaging (MRI) of the cervical spinal cord, with and without gadolinium (GD), was performed showing an intraaxial hypersignal lesion with enhancement (Figure 2). In addition, brain MRI, with and without GD revealed multiple hypersignal lesions in T2-weighted and fluid attenuated inversion recovery (FLAIR) sequences in periventricular and juxtacortical white matter and cerebellum with enhancement (Figure 3), which all were compatible with demyelinating plaques in MS and obviously distinct from non-enhancing unidentified bright objects occasionally seen symmetrically in the basal ganglia, brain stem and cerebellum in NF-1.3 Brain MRI also showed an asymptomatic large extra-axial avidly enhancing lesion at left side of the neck probably an intracranial neurofibroma or shwannoma (Figure 4); however, no biopsy was performed. Infectious etiologies such as varicella zoster virus, herpes simplex virus and cytomegalovirus were tested by polymerase chain reaction (PCR) in cerebrospinal fluid (CSF) and all were negative. Oligoclonal bands (OCB) was positive in CSF and visual evoked potential was normal bilaterally. The patient symptoms improved significantly with methylprednisolone pulse therapy with total dose of 5 grams.

Bottom Line: These two disease do not share any apparent pathological similarities.We herein present a 32-year-old woman with definite NF-1, who has recently been diagnosed with MS, which to the best of our knowledge is a rare co-occurrence.Though there are often neurologic sign and symptoms in patients with NF-1, they should not always be considered as the natural history of the disease, and other overlapped pathologies should be kept in mind, in order to not miss or postpone the efficient treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Iran University of Medical Science , Tehran, Iran.

ABSTRACT
Neurofibromatosis type 1 (NF-1) is a common hereditary neuro-cutaneous disease, with known gene mutations, that mainly involves the skin and nervous system. Multiple sclerosis (MS) is an acquired inflammatory disease in which the myelin of nerve cells in the brain and spinal cord is damaged. These two disease do not share any apparent pathological similarities. We herein present a 32-year-old woman with definite NF-1, who has recently been diagnosed with MS, which to the best of our knowledge is a rare co-occurrence. Though there are often neurologic sign and symptoms in patients with NF-1, they should not always be considered as the natural history of the disease, and other overlapped pathologies should be kept in mind, in order to not miss or postpone the efficient treatment.

No MeSH data available.


Related in: MedlinePlus