A case of hypertrophic pachymeningitis with acute on chronic headache presentation: Need for clinical vigilance in psychiatry outpatient settings

Dear Sir,

Mrs A, 29 years, young married female, belonging to a lower socioeconomic status, a migrant worker, presented to the Psychiatry Outpatient of a Tertiary Care Hospital with acute onset headache which was severe in intensity, continuous in nature, and throbbing type since 15 days with a background of chronic headache since 3 years. History of worsening headache and sensitivity to light and sounds, accompanied with nausea, were present. She reported this to be the worst headache of her life. Mrs. A reported sudden difficulty in finding words wherein even the spouse reported that he found her communication slow with difficulty in forming sentences; her supervisor reported one episode of forgetfulness of learned work over the past 1 day.

Mrs. A denied any cold and cough, earache, fever, loose stools, pain abdomen, and chills. No history of seizures, falls, weakness of any limbs, neck pain, head injury, facial asymmetry, giddiness, blurred vision, watering from eyes, any surgeries, or being on any medication was noted. No significant medical/surgical or psychiatric family history was present. Examination revealed all cranial nerves to be intact, motor system examination to be normal, deep tendon reflexes to be normal, and superficial reflexes to be normal, and sensory system and cerebellar signs were unremarkable. Kernigs and Brudenski’s test was negative. No signs of papilledema were observed on examination. Mental status examination showed difficulty in digit span test beyond 6 digits, but forward digits were intact up to 8 digits; therefore, when Mini mental status examination was done, it revealed a score of 22/30 with impairment in repetition, counting backward and spelling backward.

A provisional diagnosis as per International Classification of Diseases 10^th edition (ICD 10)[1] of G43: Migraine was made. Differential diagnosis of G44: Other headache syndromes were considered suspecting disorder of organic origin (intracranial space occupying lesion) with initiation of treatment for headache; CT scan brain was suggested with prompt referral to a neurologist.

The next day, Mrs. A reported mild to no improvement, coupled with early morning vomiting episodes, and returned with CT plain brain and MRI contrast brain reports. CT plain brain showed a heterogeneously hypodense area seen in the left parieto-temporal lobe with mass effects [Image 1], and MRI contrast brain [Image 2] showed intensely enhancing left parietal dural thickening causing mild to moderate mass effects and compression upon the left parieto-temporal lobes. A midline shift of ~ 7.2 mm toward the right side and features were suggestive of hypertrophic pachymeningitis with leptomeningitis. Following this, the patient was guided toward appropriate treatment via referral to Neurology and Neurosurgery Departments.

Image 1.

Axial section of CT plain brain showed heterogeneously hypodense area seen in left parieto-temporal lobe with mass effects

Image 2.

a-d. Axial sections of MRI brain contrast showing intensely enhancing left parietal dural thickening causing mild to moderate mass effect and compression upon the left parieto-temporal lobes. A midline shift of ~ 7.2 mm toward the right side and features were suggestive of hypertrophic pachymeningitis with leptomeningitis

Hypertrophic pachymeningitis stems from a varied etiology and is a rare clinical entity. Initial description was given by Charcot[2] and then by Naffziger and Stern.[3] Early reports attributed it to tuberculosis or syphilis. Specific etiopathogenesis of this entity remains unknown, but conjecture points to an autoimmune phenomenon or a direct result of infectious or infiltrative pathology.[3] In case detailed evaluations fail to decipher a cause, the condition of idiopathic hypertrophic cranial pachymeningitis (IHCP) is diagnosed. The most common cause for presenting clinical features is compression of anatomic structures by the meninx, thickened by inflammatory infiltration. These include headache, cranial nerve palsies, and ataxia.[4,5]

Hypertrophic cranial pachymeningitis is best identified by MRI.[6] The diagnosis is one of exclusion, eliminating all other granulomatous and infectious diseases.[7] But in most of the cases, the symptomatology and imaging characteristics are enough to come to a reasonable conclusion.[8,9]

Therefore, presentations of acute on chronic headaches with normal neurological examination findings and no papilledema with uncommon clinical diagnosis not only reinforce the need to be more vigilant in a Psychiatry OPD clinic setting but also call for vigorous radiological investigations in order to rapidly pick up disorders of organic origin.

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